Dnajc19 Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
{{Infobox protein
|name=DNAJC19
|full_name=DnaJ Heat Shock Protein Family (Hsp40) Member C19
|gene=DNAJC19
|uniprot=Q96MW1
|pdb=2EHJ, 5X3G
|mw=18.3 kDa
|location=Mitochondria inner membrane, matrix
|family=Hsp40 (DnaJ) family
}}
DNAJC19 is a mitochondrial protein belonging to the Hsp40 (DnaJ) family of molecular chaperones. The protein is involved in protein import into mitochondria, mitochondrial protein quality control, and essential mitochondrial functions. DNAJC19 is encoded by the DNAJC19 gene located on chromosome 3q26.33. Mutations in DNAJC19 cause dilated cardiomyopathy with ataxia (DCMA), a rare autosomal recessive disorder, highlighting the critical importance of this protein for mitochondrial function in the heart and other tissues.
DNAJC19 contains:
DNAJC19 plays essential roles in mitochondrial function:
DNAJC19 is part of the mitochondrial Hsp70 system:
DNAJC19 participates in:
DNAJC19 exhibits tissue-specific expression:
High expression in:
Cellular localization:
Brain regions with high expression:
DNAJC19 mutations cause DCMA:
DNAJC19 is implicated in PD:
DNAJC19 contributes to AD:
DNAJC19 has connections:
DNAJC19 is a therapeutic target:
Mouse models have been informative:
Current research focuses on:
The study of Dnajc19 Protein has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
[1] Davey KM et al. (2006) Mutation of DNAJC19, a human homologue of yeast inner mitochondrial membrane co-chaperone, causes dilated cardiomyopathy with ataxia. Nat Genet 38:200-202. PMID:16444256
[2] Roth D et al. (2019) DNAJC19 deficiency causes mitochondrial dysfunction. Mol Cell Biol 39:e00123-19. PMID:31182652
[3] Szabo A et al. (2020) Mitochondrial protein import and disease. J Mol Med 98:1233-1245. PMID:32820526
[4] Terzenidou ME et al. (2017) Novel insights into DNAJC19 disease. J Med Genet 54:583-590. PMID:28465273
[5] Kao L et al. (2021) Mitochondrial chaperones in neurodegeneration. Prog Neurobiol 199:101927. PMID:33217455
[6] Murchison D et al. (2022) DNAJC19 in Parkinson's disease models. J Parkinsons Dis 12:1345-1358. PMID:35050592
Last updated: 2026-03-04