AbstractPompe disease (PD) is a rare autosomal recessive glycogen storage disorder that causes proximal muscle weakness and loss of respiratory function. While enzyme replacement therapy (ERT) is the only effective treatment, biomarkers for disease monitoring are scarce. Following ex vivo biomarker validation in phantom studies, we apply multispectral optoacoustic tomography (MSOT), a laser- and ultrasound-based non-invasive imaging approach, in a clinical trial (NCT05083806) to image the biceps muscles of 10 late-onset PD (LOPD) patients and 10 matched healthy controls.

摘要龐貝?。≒D）是一種罕見(jiàn)的常染色體隱性糖原貯積病，可導(dǎo)致近端肌肉無(wú)力和呼吸功能喪失。雖然酶替代療法（ERT）是唯一有效的治療方法，但用于疾病監(jiān)測(cè)的生物標(biāo)志物卻很少。在體模研究中進(jìn)行離體生物標(biāo)志物驗(yàn)證后，我們?cè)谂R床試驗(yàn)（NCT05083806）中應(yīng)用多光譜光聲斷層掃描（MSOT），一種基于激光和超聲波的非侵入性成像方法，對(duì)10例遲發(fā)性PD（LOPD）患者和10例相匹配的健康對(duì)照者的肱二頭肌進(jìn)行成像。

MSOT is compared with muscle magnetic resonance imaging (MRI), ultrasound, spirometry, muscle testing and quality of life scores. Next, results are validated in an independent LOPD patient cohort from a second clinical site. Our study demonstrates that MSOT enables imaging of subcellular disease pathology with increases in glycogen/water, collagen and lipid signals, providing higher sensitivity in detecting muscle degeneration than current methods.

MSOT與肌肉磁共振成像（MRI），超聲波，肺活量測(cè)定，肌肉測(cè)試和生活質(zhì)量評(píng)分進(jìn)行比較。接下來(lái)，在第二個(gè)臨床站點(diǎn)的獨(dú)立LOPD患者隊(duì)列中驗(yàn)證結(jié)果。我們的研究表明，MSOT可以通過(guò)增加糖原/水，膠原蛋白和脂質(zhì)信號(hào)來(lái)對(duì)亞細(xì)胞疾病病理學(xué)進(jìn)行成像，從而比目前的方法在檢測(cè)肌肉變性方面提供更高的靈敏度。

This translational approach suggests implementation in the complex care of these rare disease patients..

這種轉(zhuǎn)化方法建議在這些罕見(jiàn)疾病患者的復(fù)雜護(hù)理中實(shí)施。。

IntroductionPompe disease (PD) is a rare, autosomal-recessive metabolic myopathy caused by mutations in the gene that encodes for acid alpha-glucosidase (GAA)1,2,3. Regularly, GAA catalyzes the hydrolysis of glycogen to glucose, but in PD, its impaired activity results in a generalized build-up of glycogen in metabolic active organs, such as heart, muscle and liver4,5.

簡(jiǎn)介龐貝病（PD）是一種罕見(jiàn)的常染色體隱性代謝性肌病，由編碼酸性α-葡萄糖苷酶（GAA）1,2,3的基因突變引起。通常，GAA催化糖原水解為葡萄糖，但在PD中，其活性受損導(dǎo)致代謝活性器官（如心臟，肌肉和肝臟）中糖原的普遍積累4,5。

The disease progress is variable in age of onset, severity of organ involvement and degree of myopathy6. There is a differentiation in infantile (IOPD) and late-onset (LOPD) forms based on cardiac involvement, age of onset and residual enzyme activity7. IOPD patients may have less than 1% GAA activity, therefore, quickly develop severe symptoms, such as cardiac involvement, resulting in a high mortality rate by year one if untreated1,8.

疾病的進(jìn)展因發(fā)病年齡，器官受累的嚴(yán)重程度和肌病程度而異6。根據(jù)心臟受累，發(fā)病年齡和殘留酶活性，嬰兒（IOPD）和遲發(fā)（LOPD）形式存在差異7。IOPD患者的GAA活性可能低于1%，因此，如果不治療，很快就會(huì)出現(xiàn)嚴(yán)重癥狀，例如心臟受累，導(dǎo)致第一年的死亡率很高1,8。

Children and adults with LOPD have residual enzyme activity below 30%, leading to more slowly progressive limb-girdle type weakness and respiratory insufficiency9,10. Replacement therapies (ERT) are available, leading to a slower progression of cardiac and musculoskeletal involvement, prevention of deterioration of pulmonary function and increasing survival11,12,13,14.

。替代療法（ERT）是可用的，導(dǎo)致心臟和肌肉骨骼受累的進(jìn)展較慢，預(yù)防肺功能惡化并提高生存率11,12,13,14。

However, an early initiation of treatment may positively impact the overall treatment response15.The diagnosis of PD is usually established by confirmation of GAA deficiency, and confirmed by genetic testing16,17. Furthermore, PD patients require regular clinical follow-up monitoring, especially to assess the response to ERT8,9,17,18,19,20.

然而，早期開(kāi)始治療可能會(huì)對(duì)整體治療反應(yīng)產(chǎn)生積極影響15。PD的診斷通常通過(guò)確認(rèn)GAA缺乏來(lái)確定，并通過(guò)基因檢測(cè)來(lái)證實(shí)16,17。此外，PD患者需要定期進(jìn)行臨床隨訪監(jiān)測(cè)，特別是評(píng)估對(duì)ERT8,9,17,18,19,20的反應(yīng)。

While rapid determination of GAA in dried blood spots is possible, enzymatic analysis is unable to discriminate between patients with PD and those individuals harboring pseudo deficiency mutations. In this regard, a tetraglucose oligomer (Glc(4)) in the urine and maltotet.

雖然可以快速測(cè)定干血斑中的GAA，但酶分析無(wú)法區(qū)分PD患者和攜帶偽缺陷突變的個(gè)體。在這方面，尿液和麥芽糖中的四葡萄糖低聚物（Glc（4））。

The raw (individual, identifiable patient) data are protected and are not available due to data privacy laws. Data sharing requests will be considered on a case-by-case basis. The processed pseudonymized imaging data can be accessed upon request and within the framework of legal regulations from the corresponding author (equivalent purposes to those for which the patients grant their consent to use the data).

原始（個(gè)人，可識(shí)別的患者）數(shù)據(jù)受到保護(hù)，并且由于數(shù)據(jù)隱私法而不可用。數(shù)據(jù)共享請(qǐng)求將根據(jù)具體情況予以考慮?？梢愿鶕?jù)要求并在相應(yīng)作者的法律法規(guī)框架內(nèi)訪問(wèn)處理后的假名成像數(shù)據(jù)（與患者同意使用數(shù)據(jù)的目的相同）。

Access is granted directly after publication for 36 months. The contact is ki-forschung@uk-erlangen.de, and response to request will be provided within 4–6 weeks. The data will be available for 3 months. The remaining data of this study are provided in the Supplementary Information and Source Data file.

出版36個(gè)月后直接授予訪問(wèn)權(quán)限。聯(lián)系人是ki-forschung@uk-erlangen.de，并將在4-6周內(nèi)對(duì)請(qǐng)求做出響應(yīng)。數(shù)據(jù)將持續(xù)3個(gè)月。這項(xiàng)研究的其余數(shù)據(jù)在補(bǔ)充信息和源數(shù)據(jù)文件中提供。

The study protocols and the statistical analysis plan are provided with this manuscript in the Supplementary information file. Source data are provided with this paper..

。本文提供了源數(shù)據(jù)。。

ReferencesKishnani, P. S. et al. Pompe disease diagnosis and management guideline. Genet. Med. 8, 267–288 (2006).Article

參考Kishnani，P.S.等人的龐貝病診斷和管理指南。基因。醫(yī)學(xué)8267-288（2006）。文章

Kroos, M. et al. Update of the Pompe disease mutation database with 107 sequence variants and a format for severity rating. Hum. Mutat. 29, E13–E26 (2008).Article

Kroos，M。等人。用107個(gè)序列變體和嚴(yán)重程度評(píng)級(jí)格式更新龐貝病突變數(shù)據(jù)庫(kù)。嗯。變異。29，E13–E26（2008）。文章

Kohler, L., Puertollano, R. & Raben, N. Pompe disease: from basic science to therapy. Neurotherapeutics 15, 928–942 (2018).Article

Kohler，L.，Puertollano，R。＆Raben，N。Pompe病：從基礎(chǔ)科學(xué)到治療。神經(jīng)治療學(xué)15928-942（2018）。文章

Pompe, J. C. Over idiopathische hypertrophie van het hart. Ned. Tijdschr. Geneeskd. 76, 304–311 (1932).

Pompe，J.C.關(guān)于特發(fā)性心臟肥大。奈德。痊愈了。76, 304–311 (1932).

Hers, H. G. α-Glucosidase deficiency in generalized glycogen-storage disease (Pompe’s disease). Biochem. J. 86, 11–16 (1963).Article

Hers，H.G。廣義糖原貯積?。嬝愂喜。┲械摩?葡萄糖苷酶缺乏癥。生物化學(xué)。J、 86，11-16（1963）。文章

van den Hout, H. M. et al. The natural course of infantile Pompe’s disease: 20 original cases compared with 133 cases from the literature. Pediatrics 112, 332–340 (2003).Article

van den Hout，H.M.等人，《嬰兒龐貝氏病的自然病程：20例原始病例，而文獻(xiàn)中有133例。兒科112332-340（2003）。文章

van der Ploeg, A. T. & Reuser, A. J. Pompe’s disease. Lancet 372, 1342–1353 (2008).Article

van der Ploeg，A.T。和Reuser，A.J.龐貝氏病。柳葉刀3721342-1353（2008）。文章

Kishnani, P. S. & Howell, R. R. Pompe disease in infants and children. J. Pediatr. 144, S35–S43 (2004).Article

Kishnani，P.S。＆Howell，R.R。嬰兒和兒童的龐貝病。J、 。144，S35–S43（2004）。文章

Cupler, E. J. et al. Consensus treatment recommendations for late-onset Pompe disease. Muscle Nerve 45, 319–333 (2012).Article

Cupler，E.J.等人。遲發(fā)性龐貝病的共識(shí)治療建議。肌肉神經(jīng)45319-333（2012）。文章

Preisler, N. et al. Late-onset Pompe disease is prevalent in unclassified limb-girdle muscular dystrophies. Mol. Genet. Metab. 110, 287–289 (2013).Article

Preisler，N。等人。遲發(fā)性龐貝氏癥在未分類的肢帶型肌營(yíng)養(yǎng)不良癥中很普遍。分子遺傳學(xué)。代謝。110287-289（2013）。文章

van der Ploeg, A. T. et al. A randomized study of alglucosidase alfa in late-onset Pompe’s disease. N. Engl. J. Med. 362, 1396–1406 (2010).Article

van der Ploeg，A.T.等人對(duì)阿爾法葡糖苷酶在晚發(fā)性龐貝氏病中的隨機(jī)研究。N、 英語(yǔ)。J、 醫(yī)學(xué)3621396-1406（2010）。文章

Khan, A. A. et al. Higher dosing of alglucosidase alfa improves outcomes in children with Pompe disease: a clinical study and review of the literature. Genet. Med. 22, 898–907 (2020).Article

Khan，A.A.等人。更高劑量的α-葡糖苷酶可改善龐貝氏癥兒童的預(yù)后：臨床研究和文獻(xiàn)綜述?；颉ａt(yī)學(xué)雜志22898-907（2020）。文章

Schoser, B. et al. Survival and long-term outcomes in late-onset Pompe disease following alglucosidase alfa treatment: a systematic review and meta-analysis. J. Neurol. 264, 621–630 (2017).Article

Schoser，B。等人。α-葡萄糖苷酶治療后遲發(fā)性龐貝病的生存和長(zhǎng)期預(yù)后：系統(tǒng)評(píng)價(jià)和薈萃分析。J、 神經(jīng)病學(xué)。264621-630（2017）。文章

Schoser, B. et al. Safety and efficacy of cipaglucosidase alfa plus miglustat versus alglucosidase alfa plus placebo in late-onset Pompe disease (PROPEL): an international, randomised, double-blind, parallel-group, phase 3 trial. Lancet Neurol. 20, 1027–1037 (2021).Article

Schoser，B.等人，《西格列糖苷酶α加米格司他與葡糖苷酶α加安慰劑治療遲發(fā)性龐貝?。≒ROPEL）的安全性和有效性：一項(xiàng)國(guó)際隨機(jī)雙盲平行組3期臨床試驗(yàn)》。柳葉刀神經(jīng)學(xué)。201027-1037（2021）。文章

Bolano-Diaz, C. & Diaz-Manera, J. Therapeutic options for the management of Pompe disease: current challenges and clinical evidence in therapeutics and clinical risk management. Ther. Clin. Risk Manag. 18, 1099–1115 (2022).Article

Bolano-Diaz，C。＆Diaz-Manera，J。龐貝病管理的治療選擇：治療學(xué)和臨床風(fēng)險(xiǎn)管理中的當(dāng)前挑戰(zhàn)和臨床證據(jù)。他們。臨床。風(fēng)險(xiǎn)管理。181099-1115（2022）。文章

Case, L. E., Beckemeyer, A. A. & Kishnani, P. S. Infantile Pompe disease on ERT: update on clinical presentation, musculoskeletal management, and exercise considerations. Am. J. Med. Genet. C Semin. Med. Genet. 160C, 69–79 (2012).Article

Case，L.E.，Beckemeyer，A.A。＆Kishnani，P.S。嬰兒龐貝氏癥的ERT：臨床表現(xiàn)，肌肉骨骼管理和運(yùn)動(dòng)考慮的最新情況。美國(guó)醫(yī)學(xué)雜志Genet。C半成品。Genet醫(yī)學(xué)院。160C，69-79（2012）。文章

van der Ploeg, A. T. et al. European consensus for starting and stopping enzyme replacement therapy in adult patients with Pompe disease: a 10-year experience. Eur. J. Neurol. 24, 768-e31 (2017).PubMed

van der Ploeg，A.T.等人，《歐洲關(guān)于開(kāi)始和停止龐貝氏癥成年患者酶替代療法的共識(shí)：10年經(jīng)驗(yàn)》。歐洲神經(jīng)病學(xué)雜志。24768-e31（2017）。PubMed出版社

Tarnopolsky, M. et al. Pompe disease: diagnosis and management. Evidence-based guidelines from a Canadian expert panel. Can. J. Neurol. Sci. 43, 472–485 (2016).Article

Tarnopolsky，M。等人，《龐貝?。涸\斷和治療》。加拿大專家小組的循證指南?？梢浴、 神經(jīng)病學(xué)?？茖W(xué)。43472-485（2016）。文章

Llerena Junior, J. C. et al. Guidelines for the diagnosis, treatment and clinical monitoring of patients with juvenile and adult Pompe disease. Arq. Neuropsiquiatr. 74, 166–176 (2016).Article

Llerena Junior，J.C.等人，《青少年和成人龐貝病患者的診斷、治療和臨床監(jiān)測(cè)指南》。阿Q。神經(jīng)科學(xué)。74166-176（2016）。文章

MENA Pompe Working Group. Diagnosis and treatment of late-onset Pompe disease in the Middle East and North Africa region: consensus recommendations from an expert group. BMC Neurol. 15, 205 (2015).Article

中東和北非龐貝地區(qū)工作組。中東和北非地區(qū)遲發(fā)性龐貝病的診斷和治療：專家組的共識(shí)建議。BMC神經(jīng)病學(xué)。。文章

Manwaring, V. et al. Urine analysis of glucose tetrasaccharide by HPLC; a useful marker for the investigation of patients with Pompe and other glycogen storage diseases. J. Inherit. Metab. Dis. 35, 311–316 (2012).Article

Manwaring，V。等人。通過(guò)HPLC對(duì)葡萄糖四糖進(jìn)行尿液分析；用于調(diào)查龐貝氏癥和其他糖原貯積病患者的有用標(biāo)志物。J、 繼承.Metab。Dis。35311-316（2012）。文章

Young, S. P. et al. Long-term monitoring of patients with infantile-onset Pompe disease on enzyme replacement therapy using a urinary glucose tetrasaccharide biomarker. Genet. Med. 11, 536–541 (2009).Article

?；?。醫(yī)學(xué)雜志11536-541（2009）。文章

An, Y. et al. Glucose tetrasaccharide as a biomarker for monitoring the therapeutic response to enzyme replacement therapy for Pompe disease. Mol. Genet. Metab. 85, 247–254 (2005).Article

。分子遺傳學(xué)。代謝。85247-254（2005）。文章

Figueroa-Bonaparte, S. et al. Quantitative muscle MRI to follow up late onset Pompe patients: a prospective study. Sci. Rep. 8, 10898 (2018).Article

Figueroa Bonaparte，S.等人。定量肌肉MRI隨訪晚發(fā)性龐貝病患者：一項(xiàng)前瞻性研究?？茖W(xué)。代表810898（2018）。文章

Figueroa-Bonaparte, S. et al. Muscle MRI findings in childhood/adult onset Pompe disease correlate with muscle function. PLoS ONE 11, e0163493 (2016).Article

Figueroa Bonaparte，S.等人。兒童/成人發(fā)病的龐貝氏癥的肌肉MRI表現(xiàn)與肌肉功能相關(guān)。PLoS ONE 11，e0163493（2016）。文章

Rehmann, R. et al. Diffusion tensor imaging reveals changes in non-fat infiltrated muscles in late onset Pompe disease. Muscle Nerve 62, 541–549 (2020).Article

Rehmann，R。等人。擴(kuò)散張量成像揭示了晚發(fā)性龐貝病中非脂肪浸潤(rùn)肌肉的變化。肌肉神經(jīng)62541-549（2020）。文章

Gruhn, K. M. et al. Muscle imaging data in late-onset Pompe disease reveal a correlation between the pre-existing degree of lipomatous muscle alterations and the efficacy of long-term enzyme replacement therapy. Mol. Genet. Metab. Rep. 3, 58–64 (2015).ADS

Gruhn，K.M.等人。晚發(fā)性龐貝氏癥的肌肉成像數(shù)據(jù)揭示了脂肪瘤性肌肉改變的預(yù)先存在程度與長(zhǎng)期酶替代療法的療效之間的相關(guān)性。分子遺傳學(xué)。代謝。代表3，58-64（2015）。廣告

Regensburger, A. P. et al. Detection of collagens by multispectral optoacoustic tomography as an imaging biomarker for Duchenne muscular dystrophy. Nat. Med. 25, 1905–1915 (2019).Article

Regensburger，A.P.等人。通過(guò)多光譜光聲斷層掃描檢測(cè)膠原蛋白作為杜興氏肌營(yíng)養(yǎng)不良癥的成像生物標(biāo)志物。《自然醫(yī)學(xué)》251905-1915（2019）。文章

Regensburger, A. P., Wagner, A. L., Claussen, J., Waldner, M. J. & Knieling, F. Shedding light on pediatric diseases: multispectral optoacoustic tomography at the doorway to clinical applications. Mol. Cell Pediatr. 7, 3 (2020).Article

Regensburger，A.P.，Wagner，A.L.，Claussen，J.，Waldner，M.J。＆Knieling，F(xiàn)。揭示兒科疾病：臨床應(yīng)用門(mén)口的多光譜光聲斷層掃描。分子細(xì)胞兒科。7，3（2020年）。文章

Regensburger, A. P. et al. Multispectral optoacoustic tomography for non-invasive disease phenotyping in pediatric spinal muscular atrophy patients. Photoacoustics 25, 100315 (2022).Article

Regensburger，A.P.等人。多光譜光聲斷層掃描用于小兒脊髓性肌萎縮癥患者的非侵入性疾病表型分析。光聲學(xué)25100315（2022）。文章

Tr?ger, A. P. et al. Hybrid ultrasound and single wavelength optoacoustic imaging reveals muscle degeneration in peripheral artery disease. Photoacoustics 35, 100579 (2023).Ntziachristos, V. & Razansky, D. Molecular imaging by means of multispectral optoacoustic tomography (MSOT). Chem.

Tr?ger，A.P.等人?；旌铣暫蛦尾ㄩL(zhǎng)光聲成像揭示了外周動(dòng)脈疾病中的肌肉變性。光聲學(xué)35100579（2023）。?；瘜W(xué)。

Rev. 110, 2783–2794 (2010).Article .

修訂版1102783-2794（2010）。文章。

Buehler, A., Kacprowicz, M., Taruttis, A. & Ntziachristos, V. Real-time handheld multispectral optoacoustic imaging. Opt. Lett. 38, 1404–1406 (2013).Article

Buehler，A.，Kacprowicz，M.，Taruttis，A。＆Ntziachristos，V。實(shí)時(shí)手持式多光譜光聲成像。。利特。381404-1406（2013）。文章

Manohar, S. & Razansky, D. Photoacoustics: a historical review. Adv. Opt. Photon. 8, 586–617 (2016).Article

Manohar，S。和Razansky，D。光聲學(xué)：歷史回顧。高級(jí)選項(xiàng)。光子。8586-617（2016）。文章

Schellenberg, M. W. & Hunt, H. K. Hand-held optoacoustic imaging: a review. Photoacoustics 11, 14–27 (2018).Article

Schellenberg，M.W。和Hunt，H.K。手持式光聲成像：綜述。光聲學(xué)11,14-27（2018）。文章

Steinberg, I. et al. Photoacoustic clinical imaging. Photoacoustics 14, 77–98 (2019).Article

Steinberg，I。等人。光聲臨床成像。光聲學(xué)14,77-98（2019）。文章

Regensburger, A. P., Brown, E., Kronke, G., Waldner, M. J. & Knieling, F. Optoacoustic imaging in inflammation. Biomedicines 9, 483 (2021).Wilson, K., Homan, K. & Emelianov, S. Biomedical photoacoustics beyond thermal expansion using triggered nanodroplet vaporization for contrast-enhanced imaging.

Regensburger，A.P.，Brown，E.，Kronke，G.，Waldner，M.J。＆Knieling，F(xiàn)。炎癥中的光聲成像。生物醫(yī)學(xué)9483（2021）。Wilson，K.，Homan，K。＆Emelianov，S。生物醫(yī)學(xué)光聲學(xué)超越熱膨脹，使用觸發(fā)的納米液滴蒸發(fā)進(jìn)行對(duì)比增強(qiáng)成像。

Nat. Commun. 3, 618 (2012).Article .

Nat.普通。3618（2012）。文章。

Weber, J., Beard, P. C. & Bohndiek, S. E. Contrast agents for molecular photoacoustic imaging. Nat. Methods 13, 639–650 (2016).Article

Weber，J.，Beard，P.C。和Bohndiek，S.E。用于分子光聲成像的造影劑。自然方法13639-650（2016）。文章

Razansky, D., Buehler, A. & Ntziachristos, V. Volumetric real-time multispectral optoacoustic tomography of biomarkers. Nat. Protoc. 6, 1121–1129 (2011).Article

。自然協(xié)議。61121-1129（2011）。文章

Knieling, F. et al. Multispectral optoacoustic tomography for assessment of Crohn’s disease activity. N. Engl. J. Med. 376, 1292–1294 (2017).Article

Knieling，F(xiàn)。等人。用于評(píng)估克羅恩病活動(dòng)性的多光譜光聲斷層掃描。N、 英語(yǔ)。J、 。文章

Wagner, A. L. et al. Precision of handheld multispectral optoacoustic tomography for muscle imaging. Photoacoustics 21, 100220 (2021).Article

Wagner，A.L.等人。用于肌肉成像的手持式多光譜光聲斷層掃描的精度。光聲學(xué)2110020（2021）。文章

Danko, V. et al. Hybrid reflected-ultrasound computed tomography versus B-mode-ultrasound for muscle scoring in spinal muscular atrophy. J. Neuroimaging. 33, 393–403 (2023).Raben, N. et al. Enzyme replacement therapy in the mouse model of Pompe disease. Mol. Genet. Metab. 80, 159–169 (2003).Article .

Danko，V。等人。混合反射超聲計(jì)算機(jī)斷層掃描與B型超聲對(duì)脊髓性肌萎縮癥肌肉評(píng)分的比較。J、 神經(jīng)影像學(xué)。33393-403（2023）。Raben，N.等人。龐貝病小鼠模型中的酶替代療法。分子遺傳學(xué)。代謝。80159-169（2003）。文章。

McVie-Wylie, A. J. et al. Biochemical and pharmacological characterization of different recombinant acid alpha-glucosidase preparations evaluated for the treatment of Pompe disease. Mol. Genet. Metab. 94, 448–455 (2008).Article

McVie Wylie，A.J.等人。評(píng)估用于治療龐貝病的不同重組酸性α-葡萄糖苷酶制劑的生化和藥理學(xué)表征。分子遺傳學(xué)。代謝。94448-455（2008）。文章

Cardone, M. et al. Abnormal mannose-6-phosphate receptor trafficking impairs recombinant alpha-glucosidase uptake in Pompe disease fibroblasts. Pathogenetics 1, 6 (2008).Article

。病原學(xué)1,6（2008）。文章

Kishnani, P. S. et al. Efficacy and safety of avalglucosidase alfa in patients with late-onset Pompe disease after 97 weeks: a phase 3 randomized clinical trial. JAMA Neurol. 80, 558–567 (2023).Article

。JAMA Neurol。80558-567（2023）。文章

Raben, N., Roberts, A. & Plotz, P. H. Role of autophagy in the pathogenesis of Pompe disease. Acta Myol. 26, 45–48 (2007).CAS

Raben，N.，Roberts，A。＆Plotz，P.H。自噬在龐貝病發(fā)病機(jī)制中的作用。。26，45-48（2007）。中科院

Chien, Y. H., Hwu, W. L. & Lee, N. C. Pompe disease: early diagnosis and early treatment make a difference. Pediatr. Neonatol. 54, 219–227 (2013).Article

Chien，Y.H.，Hwu，W.L。和Lee，N.C.Pompe?。涸缙谠\斷和早期治療會(huì)有所不同。兒科。新生兒醇。54219-227（2013）。文章

Yang, C. F. et al. Very early treatment for infantile-onset Pompe disease contributes to better outcomes. J. Pediatr. 169, 174–180.e171 (2016).Article

Yang，C.F.等人。嬰兒期龐貝氏癥的早期治療有助于改善預(yù)后。J、 。169174–180.e171（2016）。文章

van Capelle, C. I. et al. The quick motor function test: a new tool to rate clinical severity and motor function in Pompe patients. J. Inherit. Metab. Dis. 35, 317–323 (2012).Article

van Capelle，C.I.等人，《快速運(yùn)動(dòng)功能測(cè)試：評(píng)估龐貝病患者臨床嚴(yán)重程度和運(yùn)動(dòng)功能的新工具》。J、 繼承.Metab。Dis。35317-323（2012）。文章

Harfouche, M. et al. Use of the patient-reported outcomes measurement information system (PROMIS(R)) to assess late-onset Pompe disease severity. J. Patient Rep. Outcomes 4, 83 (2020).Article

。J、 患者代表結(jié)果4,83（2020）。文章

van der Beek, N. A., Hagemans, M. L., van der Ploeg, A. T., van Doorn, P. A. & Merkies, I. S. The Rasch-built Pompe-specific activity (R-PAct) scale. Neuromuscul. Disord. 23, 256–264 (2013).Article

van der Beek，N.A.，Hagemans，M.L.，van der Ploeg，A.T.，van Doorn，P.A。＆Merkies，I.S。Rasch建立了龐貝比活性（R-PAct）量表。神經(jīng)肌肉?；靵y。23256-264（2013）。文章

Diaz-Manera, J. et al. Safety and efficacy of avalglucosidase alfa versus alglucosidase alfa in patients with late-onset Pompe disease (COMET): a phase 3, randomised, multicentre trial. Lancet Neurol. 20, 1012–1026 (2021).Article

Diaz-Manera，J.等人，《晚發(fā)性龐貝?。–OMET）患者中α-葡萄糖苷酶與α-葡萄糖苷酶的安全性和有效性：一項(xiàng)3期隨機(jī)多中心試驗(yàn)。柳葉刀神經(jīng)學(xué)。201012-1026（2021）。文章

Salabarria, S. M. et al. Advancements in AAV-mediated gene therapy for Pompe disease. J. Neuromuscul. Dis. 7, 15–31 (2020).Article

Salabarria，S.M.等人。AAV介導(dǎo)的龐貝病基因治療的進(jìn)展。J、 神經(jīng)肌肉。Dis。7，15-31（2020）。文章

Byrne, B. J. et al. Pompe disease gene therapy. Hum. Mol. Genet. 20, R61–R68 (2011).Article

Byrne，B.J.等人，《龐貝病基因治療》。嗯，摩爾·吉內(nèi)特。20，R61–R68（2011）。文章

Rairikar, M. V. et al. Insight into the phenotype of infants with Pompe disease identified by newborn screening with the common c.-32-13T > G “l(fā)ate-onset” GAA variant. Mol. Genet. Metab. 122, 99–107 (2017).Article

Rairikar，M.V.等人。通過(guò)新生兒篩查發(fā)現(xiàn)常見(jiàn)的c.-32-13T>G“遲發(fā)性”GAA變異，洞察龐貝病嬰兒的表型。分子遺傳學(xué)。代謝。122,99-107（2017）。文章

Fernandes, S. A. et al. Quantitative whole-body magnetic resonance imaging in children with Pompe disease: clinical tools to evaluate severity of muscle disease. JIMD Rep. 57, 94–101 (2021).Article

Fernandes，S.A.等人，《龐貝氏癥兒童的定量全身磁共振成像：評(píng)估肌肉疾病嚴(yán)重程度的臨床工具》。JIMD Rep.57，94–101（2021）。文章

Carlier, P. G. et al. Skeletal muscle quantitative nuclear magnetic resonance imaging follow-up of adult Pompe patients. J. Inherit. Metab. Dis. 38, 565–572 (2015).Article

Carlier，P.G.等人。成年龐貝患者的骨骼肌定量核磁共振成像隨訪。J、 繼承.Metab。Dis。38565-572（2015）。文章

Lollert, A. et al. Quantification of intramuscular fat in patients with late-onset Pompe disease by conventional magnetic resonance imaging for the long-term follow-up of enzyme replacement therapy. PLoS ONE 13, e0190784 (2018).Article

Lollert，A。等人。通過(guò)常規(guī)磁共振成像對(duì)遲發(fā)性龐貝病患者的肌內(nèi)脂肪進(jìn)行定量，以進(jìn)行酶替代療法的長(zhǎng)期隨訪。PLoS ONE 13，e0190784（2018）。文章

Baligand, C. et al. 13C/31P MRS metabolic biomarkers of disease progression and response to AAV delivery of hGAA in a mouse model of Pompe disease. Mol. Ther. Methods Clin. Dev. 7, 42–49 (2017).Article

Baligand，C。等人。龐貝病小鼠模型中疾病進(jìn)展的13C/31P MRS代謝生物標(biāo)志物和對(duì)AAV遞送hGAA的反應(yīng)。摩爾熱。方法臨床。德文7，42-49（2017）。文章

Beha, G. et al. FP.19 Quantification of glycogen distribution in late-onset Pompe patients using 7 Tesla C13 NMR spectroscopy. Neuromuscul. Disord. 32, S73 (2022).Article

Beha，G。等人，F(xiàn)P.19使用7特斯拉C13核磁共振波譜法定量晚發(fā)性龐貝病患者的糖原分布。神經(jīng)肌肉?；靵y。32，S73（2022）。文章

Bohndiek, S. Addressing photoacoustics standards. Nat. Photonics 13, 298–298 (2019).Article

Bohndiek，S。解決光聲學(xué)標(biāo)準(zhǔn)。《自然光子學(xué)》13298-298（2019）。文章

Assi, H. et al. A review of a strategic roadmapping exercise to advance clinical translation of photoacoustic imaging: from current barriers to future adoption. Photoacoustics 32, 100539 (2023).Article

。光聲學(xué)32100539（2023）。文章

Paulus, L. P. et al. Contrast-enhanced multispectral optoacoustic tomography for functional assessment of the gastrointestinal tract. Adv. Sci. 10, e2302562 (2023).Paulus, L. P. et al. Multispectral optoacoustic tomography of the human intestine—temporal precision and the influence of postprandial gastrointestinal blood flow.

Paulus，L.P.等人。對(duì)比增強(qiáng)多光譜光聲斷層掃描用于胃腸道功能評(píng)估。高級(jí)科學(xué)。10，e2302562（2023）。Paulus，L.P.等人。人體腸道時(shí)間精度的多光譜光聲斷層掃描和餐后胃腸道血流的影響。

Photoacoustics 30, 100457 (2023).Article .

光聲學(xué)30100457（2023）。文章。

Waldner, M. J. et al. Multispectral optoacoustic tomography in Crohn’s disease: noninvasive imaging of disease activity. Gastroenterology 151, 238–240 (2016).Article

Waldner，M.J.等人，《克羅恩病的多光譜光聲斷層掃描：疾病活動(dòng)的無(wú)創(chuàng)成像》。胃腸病學(xué)151238-240（2016）。文章

Regensburger, A. P. et al. Multispectral optoacoustic tomography enables assessment of disease activity in paediatric inflammatory bowel disease. Photoacoustics 35, 100578 (2024).Article

Regensburger，A.P.等人。多光譜光聲斷層掃描可以評(píng)估小兒炎癥性腸病的疾病活動(dòng)。光聲學(xué)35100578（2024）。文章

Tascilar, K. et al. Non-invasive metabolic profiling of inflammation in joints and entheses by multispectral optoacoustic tomography. Rheumatology 62, 841–849 (2023).Article

Tascilar，K。等人。通過(guò)多光譜光聲斷層掃描對(duì)關(guān)節(jié)和附著點(diǎn)炎癥進(jìn)行非侵入性代謝分析。風(fēng)濕病62841-849（2023）。文章

Gunther, J. S. et al. Targeting muscular hemoglobin content for classification of peripheral arterial disease by noninvasive multispectral optoacoustic tomography. JACC Cardiovasc. Imaging 16, 719–721 (2023).Article

Gunther，J.S.等人。通過(guò)無(wú)創(chuàng)多光譜光聲斷層掃描對(duì)肌肉血紅蛋白含量進(jìn)行外周動(dòng)脈疾病分類。JACC心血管。成像16719-721（2023）。文章

Stoffels, I. et al. Metastatic status of sentinel lymph nodes in melanoma determined noninvasively with multispectral optoacoustic imaging. Sci. Transl. Med. 7, 317ra199 (2015).Article

Stoffels，I。等人。用多光譜光聲成像無(wú)創(chuàng)測(cè)定黑色素瘤前哨淋巴結(jié)的轉(zhuǎn)移狀態(tài)?？茖W(xué)。翻譯。醫(yī)學(xué)雜志7317RA199（2015）。文章

Grohl, J., Schellenberg, M., Dreher, K. & Maier-Hein, L. Deep learning for biomedical photoacoustic imaging: a review. Photoacoustics 22, 100241 (2021).Article

Grohl，J.，Schellenberg，M.，Dreher，K。＆Maier-Hein，L。生物醫(yī)學(xué)光聲成像的深度學(xué)習(xí)：綜述。。文章

Podsiadlo, D. & Richardson, S. The timed “Up & Go”: a test of basic functional mobility for frail elderly persons. J. Am. Geriatr. Soc. 39, 142–148 (1991).Article

Podsiadlo，D。和Richardson，S。定時(shí)“起來(lái)”：對(duì)體弱老年人基本功能活動(dòng)的測(cè)試。J、 我是老年人。Soc.39142–148（1991）。文章

ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am. J. Respir. Crit. Care Med. 166, 111–117 (2002).Heckmatt, J., Rodillo, E., Doherty, M., Willson, K. & Leeman, S. Quantitative sonography of muscle.

ATS臨床肺功能實(shí)驗(yàn)室熟練程度標(biāo)準(zhǔn)委員會(huì)。ATS聲明：六分鐘步行測(cè)試指南。Am.J.Respir。暴擊。護(hù)理醫(yī)學(xué)166111-117（2002）。Heckmatt，J.，Rodillo，E.，Doherty，M.，Willson，K。＆Leeman，S。肌肉定量超聲檢查。

J. Child Neurol. 4, S101–S106 (1989).Article .

J、 兒童神經(jīng)病學(xué)。4，S101–S106（1989）。文章。

Heckmatt, J. Z., Leeman, S. & Dubowitz, V. Ultrasound imaging in the diagnosis of muscle disease. J. Pediatr. 101, 656–660 (1982).Article

Heckmatt，J.Z.，Leeman，S。＆Dubowitz，V。超聲成像在肌肉疾病診斷中的應(yīng)用。J、 。101656-660（1982）。文章

Waldner, M. J. et al. Multispectral optoacoustic tomography in Crohn’s disease: non-invasive imaging of disease activity. Gastroenterology 151, 238–240 (2016).Günther, J. S. et al. Targeting muscular hemoglobin content for classification of peripheral arterial disease by noninvasive multispectral optoacoustic tomography.

Waldner，M.J.等人，《克羅恩病的多光譜光聲斷層掃描：疾病活動(dòng)的無(wú)創(chuàng)成像》。胃腸病學(xué)151238-240（2016）。Günther，J.S.等人。通過(guò)無(wú)創(chuàng)多光譜光聲斷層掃描針對(duì)肌肉血紅蛋白含量進(jìn)行外周動(dòng)脈疾病分類。

JACC Cardiovasc. Imaging 16, 719–721 (2023).Download referencesAcknowledgementsThe research was supported by a research Grant from Sanofi Aventis (SGZ201912542) for A.L.W., R.R., R.T. and F.K. and an EU-IMI2 JU research grant (www.screen4care.eu) under the grant agreement No. 101034427 for V.D., S.M., J.Z., F.A., R.T., A.L.W., R.R. and F.K.

JACC心血管。成像16719-721（2023）。下載參考文獻(xiàn)致謝該研究得到了賽諾菲-安萬(wàn)特（SGZ201912542）的研究資助。五十、 W.，R.R.，R。T、 。

The JU receives support from the European Union’s Horizon 2020 research and innovation programme and European Federation of Pharmaceutical Industries and Association. Further support was provided by the European Research Council under the European Union Horizon H2020 program (ERC Starting Grant No. 101115742-IseeG) for F.K and an Else Kr?ner Excellence Fellowship from the Else-Kr?ner Fresenius Stiftung for A.P.R.

JU得到了歐盟地平線2020研究與創(chuàng)新計(jì)劃和歐洲制藥工業(yè)與協(xié)會(huì)聯(lián)合會(huì)的支持。歐洲研究理事會(huì)根據(jù)歐盟地平線H2020計(jì)劃（ERC起始資助號(hào)101115742 IseeG）為F.K提供了進(jìn)一步的支持，并為A.P.R.提供了Else Kr?ner Fresenius Stiftung的Else Kr?ner卓越獎(jiǎng)學(xué)金。

A.L.W. was supported by the Rahel Hirsch Program scholarship from the Charité University Hospital Berlin. The present work was performed in (partial) fulfillment of the requirements for obtaining the degree “Dr. med.” for L.T. The sponsors of the study had no influence on study design, data collection and analysis or manuscript writing.FundingOpen Access funding enabled and organized by Projekt DEAL.Author informationAuthor notesThese authors contributed equally: Alexandra L.

A、 L.W.得到了柏林Charité大學(xué)醫(yī)院Rahel Hirsch計(jì)劃獎(jiǎng)學(xué)金的支持。目前的工作是（部分）滿足L.T.獲得“醫(yī)學(xué)博士”學(xué)位的要求。該研究的贊助商對(duì)研究設(shè)計(jì)，數(shù)據(jù)收集和分析或手稿撰寫(xiě)沒(méi)有影響。資金開(kāi)放獲取資金由Projekt交易啟用和組織。作者信息作者注意到這些作者做出了同樣的貢獻(xiàn)：Alexandra L。

Wagner, Roman Raming, Ferdinand Knieling.Authors and AffiliationsDepartment of Pediatrics and Adolescent Medicine, University Hospital Erlangen, Friedrich-Alexander-Universit?t (FAU) Erlangen-Nürnberg, Erlangen, 91054, G.

瓦格納、羅曼·拉明、費(fèi)迪南德·克尼林。作者和附屬機(jī)構(gòu)埃爾蘭根大學(xué)醫(yī)院兒科和青少年醫(yī)學(xué)系，弗里德里?！啔v山大大學(xué)（FAU）埃爾蘭根-紐倫堡，埃爾蘭根，91054，G。

PubMed Google ScholarJana ZschüntzschView author publicationsYou can also search for this author in

PubMed谷歌獎(jiǎng)學(xué)金Jana Zschüntzsch查看作者出版物您也可以在

PubMed Google ScholarStefanie MeyerView author publicationsYou can also search for this author in

PubMed Google ScholarStefanie MeyerView作者出版物您也可以在

PubMed Google ScholarAlica StobbeView author publicationsYou can also search for this author in

PubMed Google ScholarAlica StobbeView作者出版物您也可以在

PubMed Google ScholarHannah BruexView author publicationsYou can also search for this author in

PubMed Google ScholarHannah BruexView作者出版物您也可以在

PubMed Google ScholarAdrian P. RegensburgerView author publicationsYou can also search for this author in

PubMed Google ScholarAdrian P.Regensburgeview作者出版物您也可以在

PubMed Google ScholarMerle Cla?enView author publicationsYou can also search for this author in

PubMed Google ScholarMerle Cla?enView作者出版物您也可以在

PubMed Google ScholarFrauke AlvesView author publicationsYou can also search for this author in

PubMed Google ScholarFrauke AlvesView作者出版物您也可以在

PubMed Google ScholarJ?rg JüngertView author publicationsYou can also search for this author in

PubMed Google ScholarJ?rg JüngertView作者出版物您也可以在

PubMed Google ScholarUlrich RotherView author publicationsYou can also search for this author in

PubMed Google ScholarUlrich RotherView作者出版物您也可以在

PubMed Google ScholarYi LiView author publicationsYou can also search for this author in

PubMed Google ScholarYi LiView作者出版物您也可以在

PubMed Google ScholarVera DankoView author publicationsYou can also search for this author in

PubMed Google ScholarVera DankoView作者出版物您也可以在

PubMed Google ScholarWerner LangView author publicationsYou can also search for this author in

PubMed Google ScholarWerner LangView作者出版物您也可以在

PubMed Google ScholarMatthias TürkView author publicationsYou can also search for this author in

PubMed.；谷歌獎(jiǎng)學(xué)金Matthias TürkView作者出版物您也可以在

PubMed Google ScholarSandy SchmidtView author publicationsYou can also search for this author in

PubMed Google ScholarSandy SchmidtView作者出版物您也可以在

PubMed Google ScholarMatthias VorgerdView author publicationsYou can also search for this author in

PubMed Google ScholarMatthias VorgerdView作者出版物您也可以在

PubMed Google ScholarLara SchlaffkeView author publicationsYou can also search for this author in

PubMed Google ScholarLara SchlaffkeView作者出版物您也可以在

PubMed Google ScholarJoachim WoelfleView author publicationsYou can also search for this author in

PubMed Google ScholarJoachim WoelfleView作者出版物您也可以在

PubMed Google ScholarAndreas HahnView author publicationsYou can also search for this author in

PubMed谷歌學(xué)術(shù)評(píng)論HahnView作者出版物您也可以在

PubMed Google ScholarAlexander MenschView author publicationsYou can also search for this author in

PubMed Google ScholarAlexander MenschView作者出版物您也可以在

PubMed Google ScholarMartin WinterhollerView author publicationsYou can also search for this author in

PubMed Google ScholarMartin WinterhollerView作者出版物您也可以在

PubMed Google ScholarRegina TrollmannView author publicationsYou can also search for this author in

PubMed Google ScholarRegina TrollmannView作者出版物您也可以在

PubMed Google ScholarRafael Hei?View author publicationsYou can also search for this author in

PubMed Google Scholarafael Hei?View作者出版物您也可以在

PubMed Google ScholarAlexandra L. WagnerView author publicationsYou can also search for this author in

PubMed Google ScholarAlexandra L.WagnerView作者出版物您也可以在

PubMed Google ScholarRoman RamingView author publicationsYou can also search for this author in

PubMed Google ScholarRoman RamingView作者出版物您也可以在

PubMed Google ScholarFerdinand KnielingView author publicationsYou can also search for this author in

PubMed Google ScholarFerdinand KnielingView作者出版物您也可以在

PubMed Google ScholarContributionsL.T., A.L.W., J.Z., A.P.R., V.D., R.R., and F. K. designed, performed experiments, and clinical studies. J.J., V.D., and S.S., S.M., A.S., and H.B. performed clinical studies. J.Z., M.T., U.R., Y.L., W.L., A.H., R.T., A.M., M.W., J.W., and R.H.

PubMed谷歌學(xué)術(shù)貢獻(xiàn)l。T、 ，A.L.W.，J.Z.，A.P.R.，V.D.，R.R。和F.K.設(shè)計(jì)，進(jìn)行實(shí)驗(yàn)和臨床研究。J、 J.，V.D。和S.S.，S.M.，A.S。和H.B.進(jìn)行了臨床研究。J、 。

provided essential support to the clinical study. U.R., F.A., M.V., and L.S. provided essential materials or technical expertise. L.T., R.R., A.L.W., M.C., and F.K. analyzed and interpreted the data. F.K. conceived and supervised the project. L.T., R.R., and F.K. wrote the first draft of the manuscript.

為臨床研究提供了必要的支持。U、 R.，F(xiàn).A.，M.V.和L.S.提供了必要的材料或技術(shù)專業(yè)知識(shí)。五十、 T.，R.R.，A.L.W.，M.C。和F.K.分析并解釋了數(shù)據(jù)。F、 K.構(gòu)思并監(jiān)督了該項(xiàng)目。五十、 T.，R.R。和F.K.撰寫(xiě)了手稿的初稿。

All authors edited and approved the final draft.Corresponding authorCorrespondence to.

所有作者都編輯并批準(zhǔn)了最終草案。對(duì)應(yīng)作者對(duì)應(yīng)。

Ferdinand Knieling.Ethics declarations

費(fèi)迪南德·克尼林。道德宣言

Competing interests

相互競(jìng)爭(zhēng)的利益

A.P.R. and F.K. are co-inventors together with iThera Medical GmbH, Germany, on an EU patent application (EP 19 163 304.9) relating to a device and a method for analyzing optoacoustic data, an optoacoustic system and a computer program. F.K. is a member of the advisory board of iThera Medical GmbH, Munich, Germany.

A、 P.R.和F.K.與德國(guó)iThera Medical GmbH共同發(fā)明了一項(xiàng)歐盟專利申請(qǐng)（EP 19 163 304.9），涉及一種用于分析光聲數(shù)據(jù)、光聲系統(tǒng)和計(jì)算機(jī)程序的裝置和方法。F、 K.是德國(guó)慕尼黑iThera Medical GmbH咨詢委員會(huì)成員。

A.P.R. and F.K. received travel support from iThera Medical GmbH, Germany. A.P.R., A.L.W., and F.K. report travel support from Sanofi Aventis, Germany. A.P.R. and F.K. report lecture fees from Sanofi Genzyme. F.K. reports lecture fees from Siemens Healthcare GmbH. The other authors declare no competing interests..

A、 P.R.和F.K.獲得了德國(guó)iThera Medical GmbH的旅行支持。A、 P.R.、A.L.W.和F.K.報(bào)告了來(lái)自德國(guó)賽諾菲-安萬(wàn)特的旅行支持。A、 P.R.和F.K.報(bào)告賽諾菲基因酶的講座費(fèi)用。F、 K.報(bào)道了西門(mén)子醫(yī)療保健有限公司的講座費(fèi)用。其他作者聲明沒(méi)有利益沖突。。

Peer review information

同行評(píng)審信息

Nature Communications thanks the anonymous reviewer(s) for their contribution to the peer review of this work. A peer review file is available.

Nature Communications感謝匿名審稿人對(duì)這項(xiàng)工作的同行評(píng)審做出的貢獻(xiàn)?？梢垣@得同行評(píng)審文件。

Additional informationPublisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.Supplementary informationSupplementary InformationPeer Review FileReporting SummaryStudy protocol (Center 1, updated)Source dataSource DataRights and permissions.

Additional informationPublisher的注釋Springer Nature在已發(fā)布的地圖和機(jī)構(gòu)隸屬關(guān)系中的管轄權(quán)主張方面保持中立。。

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made.

開(kāi)放獲取本文是根據(jù)知識(shí)共享署名4.0國(guó)際許可證授權(quán)的，該許可證允許以任何媒體或格式使用，共享，改編，分發(fā)和復(fù)制，只要您對(duì)原始作者和來(lái)源給予適當(dāng)?shù)男湃危峁┲R(shí)共享許可證的鏈接，并指出是否進(jìn)行了更改。

The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder.

本文中的圖像或其他第三方材料包含在文章的知識(shí)共享許可中，除非在材料的信用額度中另有說(shuō)明。如果材料未包含在文章的知識(shí)共享許可中，并且您的預(yù)期用途不受法律法規(guī)的許可或超出許可用途，則您需要直接獲得版權(quán)所有者的許可。

To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/..

要查看此許可證的副本，請(qǐng)?jiān)L問(wèn)http://creativecommons.org/licenses/by/4.0/..

Reprints and permissionsAbout this articleCite this articleTan, L., Zschüntzsch, J., Meyer, S. et al. Non-invasive optoacoustic imaging of glycogen-storage and muscle degeneration in late-onset Pompe disease.

轉(zhuǎn)載和許可本文引用本文Tan，L.，Zschüntzsch，J.，Meyer，S。等人。遲發(fā)性龐貝病糖原儲(chǔ)存和肌肉變性的無(wú)創(chuàng)光聲成像。

Nat Commun 15, 7843 (2024). https://doi.org/10.1038/s41467-024-52143-6Download citationReceived: 09 December 2023Accepted: 26 August 2024Published: 08 September 2024DOI: https://doi.org/10.1038/s41467-024-52143-6Share this articleAnyone you share the following link with will be able to read this content:Get shareable linkSorry, a shareable link is not currently available for this article.Copy to clipboard.

《國(guó)家公社》157843（2024）。https://doi.org/10.1038/s41467-024-52143-6Download引文接收日期：2023年12月9日接收日期：2024年8月26日發(fā)布日期：2024年9月8日OI：https://doi.org/10.1038/s41467-024-52143-6Share本文與您共享以下鏈接的任何人都可以閱讀此內(nèi)容：獲取可共享鏈接對(duì)不起，本文目前沒(méi)有可共享的鏈接。復(fù)制到剪貼板。

Provided by the Springer Nature SharedIt content-sharing initiative

由Springer Nature SharedIt內(nèi)容共享計(jì)劃提供

CommentsBy submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.

。如果您發(fā)現(xiàn)有虐待行為或不符合我們的條款或準(zhǔn)則，請(qǐng)將其標(biāo)記為不合適。

相關(guān)知識(shí)

【健康】存錢(qián)不如“存肌肉”！有肌肉的人更長(zhǎng)壽！
肌肉流失和肌肉無(wú)力的原因和如何恢復(fù)
9成人不知道，老人肌肉退化太快
晚上喝無(wú)糖酸奶會(huì)胖嗎？
卵巢儲(chǔ)備功能減退的原因和表現(xiàn)
肌腱儲(chǔ)存能量 — 生物策略 — AskNature
儲(chǔ)存糖原最多的組織是（）
正確儲(chǔ)存嬰兒輔食
邱正慶~糖原貯積病小常識(shí)
糖原儲(chǔ)積?。℅SD）：病因查找與攜帶者篩查

網(wǎng)址: 晚發(fā)性Pompe病糖原儲(chǔ)存和肌肉退化的無(wú)創(chuàng)光聲成像 http://www.u1s5d6.cn/newsview1368448.html