ATPAF2

Chr 17

ATP synthase mitochondrial F1 complex assembly factor 2

Also known as: ATP12, ATP12p, LP3663, MC5DN1

This gene encodes an assembly factor for the F(1) component of the mitochondrial ATP synthase. This protein binds specifically to the F1 alpha subunit and is thought to prevent this subunit from forming nonproductive homooligomers during enzyme assembly. This gene is located within the Smith-Magenis syndrome region on chromosome 17. An alternatively spliced transcript variant has been described, but its biological validity has not been determined. [provided by RefSeq, Jul 2008]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMitochondrial complex V deficiency, nuclear type 1

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARLimitednot for standalone diagnostic reporting
0
Active trials
0
Pubs (1 yr)
P/LP submissions
P/LP missense
1.44
LOEUF
Mechanism
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.44LOEUF
pLI 0.000
Z-score 0.29
OE 0.92 (0.611.44)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.69Z-score
OE missense 0.85 (0.740.98)
142 obs / 167.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.92 (0.611.44)
00.351.4
Missense OE?0.85 (0.740.98)
00.61.4
Synonymous OE?0.91
01.21.6
LoF obs/exp: 14 / 15.2Missense obs/exp: 142 / 167.1Syn Z: 0.60

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ATPAF2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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