NDUFA13

Chr 19p13.11

NADH:ubiquinone oxidoreductase subunit A13

Aliases:: CGI-39, CDA016, GRIM-19, GRIM19, B16.6
MANE:: ENST00000507754.9

OMIM: 609435 ↗NCBI: 51079 ↗Ensembl ↗

Annotations refreshed 1 month ago.

Predicted protein structure

AlphaFold predicted structure

NDUFA13 · Q9P0J0

v62025-08-01

Predicted Aligned Error matrix for NDUFA13 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

93.9/ 100

Very high

144 residues

Confidence breakdown

Very high(≥ 90)88%
Confident(70–90)10%
Low(50–70)1%
Very low(< 50)1%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

Likely inborn error of metabolism
BIALLELIC, autosomal or pseudoautosomal
Mitochondrial disorder with complex I deficiency
BIALLELIC, autosomal or pseudoautosomal
Mitochondrial disorders
BIALLELIC, autosomal or pseudoautosomal
Possible mitochondrial disorder - nuclear genes
BIALLELIC, autosomal or pseudoautosomal
Inherited non-medullary thyroid cancer
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

Disease associations (Open Targets)

mitochondrial complex I deficiency, nuclear type 28
0.65
type 2 diabetes mellitus
0.62
diabetes mellitus
0.58
mitochondrial complex I deficiency
0.50
polycystic ovary syndrome
0.43
Thyroid Gland Oncocytic Follicular Carcinoma
0.43
gestational diabetes
0.41
obesity disorder
0.41
Insulin resistance
0.40
Obesity
0.40

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13

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)

Curated MONDO disease pages that list NDUFA13 among their top associated genes.

polycystic ovary syndromeRare

Predicted protein structure

Clinical relevance (Genomics England PanelApp)

Disease associations (Open Targets)

Protein function (UniProt)

Diseases associated with NDUFA13