PPA2

Chr 4AR

inorganic pyrophosphatase 2

Also known as: HSPC124, SCFAI, SCFI, SID6-306

The protein encoded by this gene is localized to the mitochondrion, is highly similar to members of the inorganic pyrophosphatase (PPase) family, and contains the signature sequence essential for the catalytic activity of PPase. PPases catalyze the hydrolysis of pyrophosphate to inorganic phosphate, which is important for the phosphate metabolism of cells. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

?Sudden cardiac failure, alcohol-inducedMIM #617223
AR
Sudden cardiac failure, infantileMIM #617222
AR

Clinical highlights

Gene-disease validity (ClinGen)
dilated cardiomyopathy · ARStrongappropriate for clinical testing
0
Active trials
21
Pubs (1 yr)
P/LP submissions
P/LP missense
1.27
LOEUF
Mechanism

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.27LOEUF
pLI 0.000
Z-score 0.62
OE 0.85 (0.581.27)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.43Z-score
OE missense 0.91 (0.801.03)
159 obs / 175.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.85 (0.581.27)
00.351.4
Missense OE?0.91 (0.801.03)
00.61.4
Synonymous OE?0.95
01.21.6
LoF obs/exp: 17 / 20.0Missense obs/exp: 159 / 175.2Syn Z: 0.32

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PPA2 · 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.

Search ClinicalTrials.gov →