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

Name: Mouse Anti-DNAJC19 Recombinant Antibody (3H4)
SKU: CBMAB-D1311-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

3H4

Application

ELISA, WB

Immunogen

DNAJC19 (aa20-116) partial recombinant protein.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG2a, κ

Clonality

Monoclonal Antibody

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!]

Format

Liquid

Buffer

PBS

Preservative

None

Concentration

Batch dependent

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

More Infomation

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

Zhou, J., Peng, Y., Gao, Y. C., Chen, T. Y., Li, P. C., Xu, K., ... & Ren, T. (2021). Targeting DNAJC19 overcomes tumor growth and lung metastasis in NSCLC by regulating PI3K/AKT signaling. Cancer Cell International, 21(1), 1-11.

Ren, T. (2021). Targeting DNAJC19 overcomes tumor growth and metastasis in NSCLC by regulating AKT1 signaling.

Janz, A., Chen, R., Regensburger, M., Ueda, Y., Rost, S., Klopocki, E., ... & Gerull, B. (2020). Generation of two patient-derived iPSC lines from siblings (LIBUCi001-A and LIBUCi002-A) and a genetically modified iPSC line (JMUi001-A-1) to mimic dilated cardiomyopathy with ataxia (DCMA) caused by a homozygous DNAJC19 mutation. Stem cell research, 46, 101856.

Lopez, M. R., Hamdan, F., Rezazadeh, A., Aznarez, I., Minassian, B., Cossette, P., ... & Andrade, D. (2019). Seizures, dystonia and progressive cerebellar degeneration in homozygous DNAJC19 mutation without dilated cardiomyopathy or 3-methylglutaconic aciduria (P2. 5-013).

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Isotype control

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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