Mouse Anti-NDUFA13 Recombinant Antibody (1A8) (CBMAB-G6394-LY)

Name: Mouse Anti-NDUFA13 Recombinant Antibody (1A8)
SKU: CBMAB-G6394-LY
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

1A8

Application

Immunogen

Recombinant human GRIM-19

Specificity

Human, Mouse

Antibody Isotype

IgG2a

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!]

Format

Liquid

Purity

> 95% Purity determined by SDS-PAGE.

Storage

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

More Infomation

Target

Full Name

NADH:Ubiquinone Oxidoreductase Subunit A13

Introduction

This gene encodes a subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), which functions in the transfer of electrons from NADH to the respiratory chain. The protein is required for complex I assembly and electron transfer activity. The protein binds the signal transducers and activators of transcription 3 (STAT3) transcription factor, and can function as a tumor suppressor. The human protein purified from mitochondria migrates at approximately 16 kDa. Transcripts originating from an upstream promoter and capable of expressing a protein with a longer N-terminus have been found, but their biological validity has not been determined. [provided by RefSeq, Oct 2009]

Entrez Gene ID

Human	51079
Mouse	67184

UniProt ID

Human	Q9P0J0
Mouse	Q9ERS2

Alternative Names

NADH:Ubiquinone Oxidoreductase Subunit A13; Gene Associated With Retinoic And Interferon-Induced Mortality 19 Protein; Gene Associated With Retinoic And IFN-Induced Mortality 19 Protein; NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex; 13; NADH-Ubiquinone Oxidoreductase B16.6 Subunit; Cell Death Regulatory Protein GRIM-19; Complex I B16.6 Subunit; Complex I-B16.6; CI-B16.6;

Function

Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis (PubMed:27626371).

Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (PubMed:27626371).

Involved in the interferon/all-trans-retinoic acid (IFN/RA) induced cell death. This apoptotic activity is inhibited by interaction with viral IRF1. Prevents the transactivation of STAT3 target genes. May play a role in CARD15-mediated innate mucosal responses and serve to regulate intestinal epithelial cell responses to microbes (PubMed:15753091).

Biological Process

Aerobic respiration Source: ComplexPortal
Apoptotic signaling pathway Source: UniProtKB
Cellular response to interferon-beta Source: ParkinsonsUK-UCL
Cellular response to retinoic acid Source: ParkinsonsUK-UCL
Electron transport chain Source: GOC
Extrinsic apoptotic signaling pathway Source: Ensembl
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Negative regulation of cell growth Source: UniProtKB
Negative regulation of intrinsic apoptotic signaling pathway Source: UniProtKB
Negative regulation of transcription, DNA-templated Source: UniProtKB
Positive regulation of cysteine-type endopeptidase activity involved in apoptotic process Source: ParkinsonsUK-UCL
Positive regulation of peptidase activity Source: ParkinsonsUK-UCL
Positive regulation of protein catabolic process Source: ParkinsonsUK-UCL
Protein insertion into mitochondrial inner membrane Source: UniProtKB
Reactive oxygen species metabolic process Source: UniProtKB

Cellular Location

Mitochondrion
Mitochondrion inner membrane
Nucleus
Note: Localizes mainly in the mitochondrion (PubMed:12628925). May be translocated into the nucleus upon IFN/RA treatment.

Involvement in disease

Hurthle cell thyroid carcinoma (HCTC):
A rare type of thyroid cancer accounting for only about 3-10% of all differentiated thyroid cancers. These neoplasms are considered a variant of follicular carcinoma of the thyroid and are referred to as follicular carcinoma, oxyphilic type.
Mitochondrial complex I deficiency, nuclear type 28 (MC1DN28):
A form of mitochondrial complex I deficiency, the most common biochemical signature of mitochondrial disorders, a group of highly heterogeneous conditions characterized by defective oxidative phosphorylation, which collectively affects 1 in 5-10000 live births. Clinical disorders have variable severity, ranging from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. MC1DN28 transmission pattern is consistent with autosomal recessive inheritance.

Topology

Helical: 30-51

Kaiyrzhanov, R., & NDUFA13 Study Group. (2023). Mitochondrial Complex I Deficiency Associated with Biallelic NDUFA13 Variants Lead to Leigh Syndrome (P11-9.008).

Hörberg, J., Hallbäck, B., Moreau, K., & Reymer, A. (2022). Abnormal methylation in the NDUFA13 gene promoter of breast cancer cells breaks the cooperative DNA recognition by transcription factors. QRB Discovery, 3, e23.

Johanna, H., Björn, H., Kevin, M., & Reymer, A. (2022). Methylation in NDUFA13 gene promoter disrupts communication between collaborative transcription factors–potential mechanism for onset of breast cancer. bioRxiv, 2022-06.

Liu, X. D., Li, Y. G., Wang, G. Y., Bi, Y. G., Zhao, Y., Yan, M. L., ... & Zhang, Q. Y. (2020). Metformin protects high glucose‑cultured cardiomyocytes from oxidative stress by promoting NDUFA13 expression and mitochondrial biogenesis via the AMPK signaling pathway. Molecular medicine reports, 22(6), 5262-5270.

González-Quintana, A., García-Consuegra, I., Belanger-Quintana, A., Serrano-Lorenzo, P., Lucia, A., Blázquez, A., ... & Martín, M. A. (2020). Novel NDUFA13 mutations associated with OXPHOS deficiency and leigh syndrome: a second family report. Genes, 11(8), 855.

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

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