Ay Lin Kho
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Explore the profile of Ay Lin Kho including associated specialties, affiliations and a list of published articles.
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14
Citations
695
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Recent Articles
1.
Eibach Y, Kreher S, Poetsch M, Kho A, Gaertner U, Clemen C, et al.
Sci Adv
. 2025 Jan;
11(4):eado3852.
PMID: 39841822
Protein homeostasis is crucial for maintaining cardiomyocyte (CM) function. Disruption of proteostasis results in accumulation of protein aggregates causing cardiac pathologies such as hypertrophy, dilated cardiomyopathy (DCM), and heart failure....
2.
Pearce A, Ponnam S, Holt M, Randall T, Beckingham R, Kho A, et al.
J Biol Chem
. 2023 Dec;
300(1):105511.
PMID: 38042491
Myosin binding protein-C (MyBP-C) is a multidomain protein that regulates muscle contraction. Mutations in MYBPC3, the gene encoding for the cardiac variant (henceforth called cMyBP-C), are amongst the most frequent...
3.
Tamborrini D, Wang Z, Wagner T, Tacke S, Stabrin M, Grange M, et al.
Nature
. 2023 Nov;
623(7988):863-871.
PMID: 37914933
The thick filament is a key component of sarcomeres, the basic units of striated muscle. Alterations in thick filament proteins are associated with familial hypertrophic cardiomyopathy and other heart and...
4.
Koch D, Kho A, Fukuzawa A, Alexandrovich A, Vanaanen K, Beavil A, et al.
PLoS One
. 2023 Apr;
18(4):e0284453.
PMID: 37079638
Obscurin is a giant muscle protein (>800 kDa) featuring multiple signalling domains, including an SH3-DH-PH domain triplet from the Trio-subfamily of guanosine nucleotide exchange factors (GEFs). While previous research suggests...
5.
Wang Z, Grange M, Pospich S, Wagner T, Kho A, Gautel M, et al.
Science
. 2022 Feb;
375(6582):eabn1934.
PMID: 35175800
In skeletal muscle, nebulin stabilizes and regulates the length of thin filaments, but the underlying mechanism remains nebulous. In this work, we used cryo-electron tomography and subtomogram averaging to reveal...
6.
Koch D, Alexandrovich A, Funk F, Kho A, Schmitt J, Gautel M
Cell Rep
. 2021 Jul;
36(4):109448.
PMID: 34320358
Phospholamban (PLN) is an important regulator of cardiac calcium handling due to its ability to inhibit the calcium ATPase SERCA. β-Adrenergic stimulation reverses SERCA inhibition via PLN phosphorylation and facilitates...
7.
Wang Z, Grange M, Wagner T, Kho A, Gautel M, Raunser S
Cell
. 2021 Mar;
184(8):2135-2150.e13.
PMID: 33765442
Sarcomeres are force-generating and load-bearing devices of muscles. A precise molecular picture of how sarcomeres are built underpins understanding their role in health and disease. Here, we determine the molecular...
8.
Rees M, Nikoopour R, Fukuzawa A, Kho A, Fernandez-Garcia M, Wraige E, et al.
Acta Neuropathol
. 2021 Jan;
141(3):431-453.
PMID: 33449170
Mutations in the sarcomeric protein titin, encoded by TTN, are emerging as a common cause of myopathies. The diagnosis of a TTN-related myopathy is, however, often not straightforward due to...
9.
Zaleska M, Fogl C, Kho A, Ababou A, Ehler E, Pfuhl M
PLoS One
. 2015 Dec;
10(12):e0144614.
PMID: 26656831
Ms1 (also known as STARS and ABRA) has been shown to act as an early stress response gene in processes as different as hypertrophy in skeletal and cardiac muscle and...
10.
Chauveau C, Bonnemann C, Julien C, Kho A, Marks H, Talim B, et al.
Hum Mol Genet
. 2013 Oct;
23(4):980-91.
PMID: 24105469
Core myopathies (CM), the main non-dystrophic myopathies in childhood, remain genetically unexplained in many cases. Heart disease is not considered part of the typical CM spectrum. No congenital heart defect...