Loss of mitochondrial protein CHCHD10 in skeletal muscle causes neuromuscular junction impairment

Y Xiao, J Zhang, X Shu, L Bai, W Xu… - Human molecular …, 2020 - academic.oup.com
… To further examine whether mitochondria function in skeletal muscle fibers is impaired
without CHCHD10, we checked the ATP levels in Ad-Ctrl and Ad-Cre infected CHCHD10 f/f …

CHCHD10 mutations promote loss of mitochondrial cristae junctions with impaired mitochondrial genome maintenance and inhibition of apoptosis

EC Genin, M Plutino, S Bannwarth, E Villa… - EMBO molecular …, 2016 - embopress.org
… enriched at cristae junctions but the role played by this protein in both … Here, we show that
CHCHD10 is a component of the … The protein levels of assembled CHCHD10 and CHCHD3 …

In vitro and in vivo studies of the ALS-FTLD protein CHCHD10 reveal novel mitochondrial topology and protein interactions

SR Burstein, F Valsecchi, H Kawamata… - Human molecular …, 2018 - academic.oup.com
… mutant versions of CHCHD10, but not WT, have impaired mitochondrial energy metabolism.
… However, ATD3A, a single-transmembrane IM protein located in the ER-mito junctions was …

Mitochondrial CHCHD-containing proteins: physiologic functions and link with neurodegenerative diseases

ZD Zhou, WT Saw, EK Tan - Molecular neurobiology, 2017 - Springer
proteins can lead to mitochondrial impairment and cell demise, which are associated with
a range of human disordersCHCHD10 is located in IMS and enriched at crista junctions. …

Chchd10, A Novel Bi-Organellar Regulator Of Cellular Metabolism: Implications In Neurodegeneration

N Purandare - 2018 - digitalcommons.wayne.edu
CHCHD10 from the other twin CX9C family members is the causal association of several
mutations in both proteins with a myriad of neurodegenerative disorders, in particular ALS for …

[HTML][HTML] Neurodegeneration-associated mitochondrial proteins, CHCHD2 and CHCHD10–what distinguishes the two?

A Ikeda, Y Imai, N Hattori - Frontiers in Cell and Developmental …, 2022 - frontiersin.org
… , which is found near the mitochondrial cristae junction, was reported to be resident in the …
aggregates in mitochondria, it causes mitochondrial dysfunction and activation of the PINK1-…

Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouse

EC Genin, B Madji Hounoum, S Bannwarth… - Acta …, 2019 - Springer
dysfunction can trigger motor neuron degeneration, through identification of CHCHD10
encoding a mitochondrial protein… mitochondrial dysfunction associated with CHCHD10 mutations …

[HTML][HTML] The cellular stress proteins CHCHD10 and MNRR1 (CHCHD2): Partners in mitochondrial and nuclear function and dysfunction

N Purandare, M Somayajulu, M Hüttemann… - Journal of Biological …, 2018 - ASBMB
… We show that nuclear CHCHD10 protein down-regulates the expression of genes harboring
… CXXC finger protein 5 (CXXC5). We further show that two genetic CHCHD10 disease …

[HTML][HTML] CHCHD2 and CHCHD10: Future therapeutic targets in cognitive disorder and motor neuron disorder

T Jiang, Y Wang, X Wang, J Xu - Frontiers in Neuroscience, 2022 - frontiersin.org
… CHCHD2 and/or CHCHD10 binding to various target proteins (such as OPA… impairments
and motor neuron diseases. This review focuses on the functions of CHCHD2 and/or CHCHD10

CHCHD2 and CHCHD10-related neurodegeneration: molecular pathogenesis and the path to precision therapy

MK Shammas, TH Huang… - Biochemical Society …, 2023 - portlandpress.com
… these disorders is revealing how mitochondrial dysfunction … , driven by protein misfolding
of CHCHD2 and CHCHD10 into … and CHCHD10 transiently interact with cristae junction