Last update 01 Nov 2024

DNAJC1

Last update 01 Nov 2024

Basic Info

Synonyms

DnaJ heat shock protein family (Hsp40) member C1, DnaJ homolog subfamily C member 1, DnaJ protein homolog MTJ1

+ [5]

Introduction

May modulate protein synthesis.

Drugs associated with DNAJC1

GAP-14

Target

DNAJC1

Mechanism

DNAJC1 inhibitors

Active Org.

Tangdu Hospital

Originator Org.

Tangdu Hospital

Active Indication

Glioma

Inactive Indication-

Drug Highest PhasePreclinical

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

100 Clinical Results associated with DNAJC1

100 Translational Medicine associated with DNAJC1

0 Patents (Medical) associated with DNAJC1

Literatures (Medical) associated with DNAJC1

05 Jun 2024·G3: Genes, Genomes, Genetics

A naturally occurring canine model of syndromic congenital microphthalmia

Article

Author: O'Connor, Cynthia ; Aguirre, Gustavo D ; Niggel, Jessica K ; Banjeree, Esha ; Miyadera, Keiko ; Werner, Petra ; Casal, Margret L ; Murgiano, Leonardo

AbstractIn humans, the prevalence of congenital microphthalmia is estimated to be 0.2–3.0 for every 10,000 individuals, with nonocular involvement reported in ∼80% of cases. Inherited eye diseases have been widely and descriptively characterized in dogs, and canine models of ocular diseases have played an essential role in unraveling the pathophysiology and development of new therapies. A naturally occurring canine model of a syndromic disorder characterized by microphthalmia was discovered in the Portuguese water dog. As nonocular findings included tooth enamel malformations, stunted growth, anemia, and thrombocytopenia, we hence termed this disorder Canine Congenital Microphthalmos with Hematopoietic Defects. Genome-wide association study and homozygosity mapping detected a 2 Mb candidate region on canine chromosome 4. Whole-genome sequencing and mapping against the Canfam4 reference revealed a Short interspersed element insertion in exon 2 of the DNAJC1 gene (g.74,274,883ins[T70]TGCTGCTTGGATT). Subsequent real-time PCR-based mass genotyping of a larger Portuguese water dog population found that the homozygous mutant genotype was perfectly associated with the Canine Congenital Microphthalmos with Hematopoietic Defects phenotype. Biallelic variants in DNAJC21 are mostly found to be associated with bone marrow failure syndrome type 3, with a phenotype that has a certain degree of overlap with Fanconi anemia, dyskeratosis congenita, Shwachman–Diamond syndrome, Diamond–Blackfan anemia, and reports of individuals showing thrombocytopenia, microdontia, and microphthalmia. We, therefore, propose Canine Congenital Microphthalmos with Hematopoietic Defects as a naturally occurring model for DNAJC21-associated syndromes.

01 Jun 2024·Journal of Biological Chemistry

Grp78 destabilization of infectious prions is strain-specific and modified by multiple factors including accessory chaperones and pH

Article

Author: Shoup, Daniel ; Priola, Suzette A

Lethal neurodegenerative prion diseases result from the continuous accumulation of infectious and variably protease-resistant prion protein aggregates (PrP^D) which are misfolded forms of the normally detergent soluble and protease-sensitive cellular prion protein. Molecular chaperones like Grp78 have been found to reduce the accumulation of PrP^D, but how different cellular environments and other chaperones influence the ability of Grp78 to modify PrP^D is poorly understood. In this work, we investigated how pH and protease-mediated structural changes in PrP^D from two mouse-adapted scrapie prion strains, 22L and 87V, influenced processing by Grp78 in the presence or absence of chaperones Hsp90, DnaJC1, and Stip1. We developed a cell-free in vitro system to monitor chaperone-mediated structural changes to, and disaggregation of, PrP^D. For both strains, Grp78 was most effective at structurally altering PrP^D at low pH, especially when additional chaperones were present. While Grp78, DnaJC1, Stip1, and Hsp90 were unable to disaggregate the majority of PrP^D from either strain, pretreatment of PrP^D with proteases increased disaggregation of 22L PrP^D compared to 87V, indicating strain-specific differences in aggregate structure were impacting chaperone activity. Hsp90 also induced structural changes in 87V PrP^D as indicated by an increase in the susceptibility of its n-terminus to proteases. Our data suggest that, while chaperones like Grp78, DnaJC1, Stip1, and Hsp90 disaggregate only a small fraction of PrP^D, they may still facilitate its clearance by altering aggregate structure and sensitizing PrP^D to proteases in a strain and pH-dependent manner.

01 Jan 2023·PeerJ

DNAJ heat shock protein family member C1 can regulate proliferation and migration in hepatocellular carcinoma.

Article

Author: Jiang, Li-He ; Zhang, Chao-Sheng ; Wei, De-Wei ; Wei, Wan-Shuo ; Xie, Xian-Dong ; Fan, Yu-Chun ; Meng, Zhi-Yong ; Huang, Ming-Lu

BackgroundDNAJ heat shock protein family (Hsp40) member C1(DNAJC1) is a member of the DNAJ family. Some members of the DNAJ gene family had oncogenic properties in many cancers. However, the role of DNAJC1 in hepatocellular carcinoma (HCC) was unclear.MethodsIn this study, expression and prognostic value of DNAJC1 in HCC were analyzed by bioinformatics. Quantitative real-time PCR and Western blotting were used to verify DNAJC1 expression in liver cancer cell lines. Furthermore, immunohistochemical (IHC) was used to detect DNAJC1 expression in liver cancer tissues. Subsequently, the effect of DNAJC1 on the proliferation, migration, invasion and apoptosis of HCC cells was detected by knocking down DNAJC1. Finally, gene set enrichment analysis (GSEA) was used to investigate the potential mechanism of DNAJC1 and was verified by Western blotting.ResultsDNAJC1 was highly expressed in HCC and was significantly associated with the prognosis of patients with HCC. Importantly, the proliferation, migration and invasion of Huh7 and MHCC97H cells were inhibited by the knockdown of DNAJC1 and the knockdown of DNAJC1 promoted Huh7 and MHCC97H cell apoptosis. Furthermore, compared to the negative control group, DNAJC1 knockdown in Huh7 and MHCC97H cells promoted the expression of p21, p53, p-p53(Ser20), Bax and E-cadherin proteins, while inhibiting the expression of PARP, MMP9, Vimentin, Snai1, Bcl-2 and N-cadherin proteins.ConclusionsDNAJC1 had a predictive value for the prognosis of HCC. Knockdown of DNAJC1 may inhibit HCC cell proliferation, migration and invasion and promote the HCC cell apoptosis through p53 and EMT signaling pathways.

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DNAJC1

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