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Mouse Anti-DNAJC19 Recombinant Antibody (3H4) (CBMAB-D1311-YC)

Provided herein is a Mouse monoclonal antibody, which binds to DnaJ Heat Shock Protein Family (Hsp40) Member C19 (DNAJC19). The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all DNAJC19 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
3H4
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
DNAJC19 (NP_660304, 20 a.a. ~ 116 a.a) partial recombinant protein with GST tag. The immunogen sequence: RYVLQAMKHM EPQVKQVFQS LPKSAFSGGY YRGGFEPKMT KREAALILGV SPTANKGKIR DAHRRIMLLN HPDKGGSPYI AAKINEAKDL LEGQAKK
Specificity
Human
Antibody Isotype
IgG2a, κ
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.
Epitope
aa 20-116

Target

Full Name
DnaJ (Hsp40) homolog, subfamily C, member 19
Introduction
DNAJC19 is thought to be part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. Defects in this gene are a cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA).
Entrez Gene ID
131118
UniProt ID
Q96DA6
Alternative Names
DnaJ Heat Shock Protein Family (Hsp40) Member C19; DnaJ (Hsp40) Homolog, Subfamily C, Member 19; TIMM14; TIM14; Mitochondrial Import Inner Membrane Translocase Subunit TIM14; DnaJ-Like Protein Subfamily C Member 19; DnaJ Homolog Subfamily C Member 19; Homolog Of Yeast TIM14; PAM18;
Function
Mitochondrial co-chaperone which forms a complex with prohibitins to regulate cardiolipin remodeling (By similarity).

May be a component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity).
Biological Process
Genitalia development Source: UniProtKB
Protein folding Source: UniProtKB
Protein import into mitochondrial matrix Source: GO_Central
Protein targeting to mitochondrion Source: UniProtKB
Regulation of cardiolipin metabolic process Source: UniProtKB
Visual perception Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
3-methylglutaconic aciduria 5 (MGCA5):
An autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure and significant increases in urine organic acids, particularly 3-methylglutaconic acid and 3-methylglutaric acid.
Topology
Mitochondrial intermembrane: 2-3
Helical: 4-24
Mitochondrial matrix: 25-116
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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