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Mouse Anti-DNAJC5 Recombinant Antibody (CBMY-C0180) (CBMAB-V208-C0183-YY)

This product is mouse antibody that recognizes DNAJC5. The antibody CBMY-C0180 can be used for immunoassay techniques such as: ELISA, WB
See all DNAJC5 antibodies

Summary

Host Animal
Mouse
Specificity
P. vivax
Clone
CBMY-C0180
Antibody Isotype
IgG1
Application
ELISA, WB

Basic Information

Specificity
P. vivax
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
DnaJ Heat Shock Protein Family (Hsp40) Member C5; DnaJ (Hsp40) Homolog, Subfamily C, Member; Ceroid-Lipofuscinosis Neuronal Protein; CLN4; Ceroid-Lipofuscinosis, Neuronal 4 (Kufs Disease) 2
DnaJ Homolog Subfamily C Member; Cysteine String Protein Alpha 3; Cysteine String Protein 4
Introduction
DNAJC5 (DnaJ Heat Shock Protein Family (Hsp40) Member C5) is a Protein Coding gene. Diseases associated with DNAJC5 include Ceroid Lipofuscinosis, Neuronal, 4B, Autosomal Dominant and Ceroid Storage Disease.
Function
Acts as a general chaperone in regulated exocytosis (By similarity).

Acts as a co-chaperone for the SNARE protein SNAP-25 (By similarity).

Involved in the calcium-mediated control of a late stage of exocytosis (By similarity).

May have an important role in presynaptic function. May be involved in calcium-dependent neurotransmitter release at nerve endings (By similarity).
Biological Process
Chaperone-mediated protein folding Source: Ensembl
Exocytosis Source: ParkinsonsUK-UCL
Negative regulation of neuron apoptotic process Source: Ensembl
Regulated exocytosis Source: ParkinsonsUK-UCL
Regulation of synaptic vesicle cycle Source: Ensembl
Synaptic vesicle exocytosis Source: ParkinsonsUK-UCL
Cellular Location
Cell membrane; Cytosol; Membrane; Chromaffin granule membrane; Melanosome. The association with membranes is regulated by palmitoylation (By similarity). Identified by mass spectrometry in melanosome fractions from stage I to stage IV (PubMed:17081065).
Involvement in disease
Ceroid lipofuscinosis, neuronal, 4B (CLN4B):
An adult-onset neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. CLN4B has no visual involvement and is characterized by seizures and other neurologic symptoms.
PTM
Ser-10 phosphorylation induces an order-to-disorder transition triggering the interaction with Lys-58 (PubMed:27452402). This conformational switch modulates DNAJC5's cellular functions by reducing binding to syntaxin and synaptogamin without altering HSC70 interactions (PubMed:27452402).
Palmitoylated. Could be palmitoylated by DHHC3, DHHC7, DHHC15 and DHHC17. Palmitoylation occurs probably in the cysteine-rich domain and regulates DNAJC5 membrane attachment.

Lee, J., Xu, Y., Saidi, L., Xu, M., Zinsmaier, K., & Ye, Y. (2022). Abnormal triaging of misfolded proteins by adult neuronal ceroid lipofuscinosis-associated DNAJC5/CSPα mutants causes lipofuscin accumulation. Autophagy, 1-20.

Wu, S., Sirkis, D. W., & Schekman, R. (2022). Unconventional secretion of α-synuclein mediated by palmitoylated DNAJC5 oligomers. BioRxiv.

Huang, Q., Zhang, Y. F., Li, L. J., Dammer, E. B., Hu, Y. B., Xie, X. Y., ... & Ren, R. J. (2022). Adult-Onset Neuronal Ceroid Lipofuscinosis With a Novel DNAJC5 Mutation Exhibits Aberrant Protein Palmitoylation. Frontiers in aging neuroscience, 14.

Wang, H., Luo, J., Tian, X., Xu, L., Zhai, Z., Cheng, M., ... & Luo, S. (2021). DNAJC5 promotes hepatocellular carcinoma cells proliferation though regulating SKP2 mediated p27 degradation. Biochimica et Biophysica Acta (BBA)-Molecular Cell Research, 1868(6), 118994.

Faruq, R. N., D’Silva, P., Lau, F. D., Zhao, C., & Majumdar, S. (2021). Early-Onset Vascular Dementia in a 43-Year-Old Man with Accelerated Atherosclerotic Disease, Elevated Lipoprotein (a), and a Missense DNAJC5 Variant with Potential Association to Adult-Onset Ceroid Lipofuscinosis. Case reports in neurology, 13, 565-571.

Jedličková, I., Cadieux-Dion, M., Přistoupilová, A., Stránecký, V., Hartmannová, H., Hodaňová, K., ... & Kmoch, S. (2020). Autosomal-dominant adult neuronal ceroid lipofuscinosis caused by duplication in DNAJC5 initially missed by Sanger and whole-exome sequencing. European Journal of Human Genetics, 28(6), 783-789.

Xu, Y., Cui, L., Dibello, A., Wang, L., Lee, J., Saidi, L., ... & Ye, Y. (2018). DNAJC5 facilitates USP19-dependent unconventional secretion of misfolded cytosolic proteins. Cell discovery, 4(1), 1-18.

Valenzuela-Villatoro, M., García-Junco-Clemente, P., Nieto-González, J. L., & Fernández-Chacón, R. (2018). Presynaptic neurodegeneration: CSP-α/DNAJC5 at the synaptic vesicle cycle and beyond. Current Opinion in Physiology, 4, 65-69.

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For research use only. Not intended for any clinical use.

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