PANK4

Chr 1AD

pantothenate kinase 4 (inactive)

Also known as: CTRCT49

This gene encodes a protein belonging to the pantothenate kinase family. Pantothenate kinase is a key regulatory enzyme in the biosynthesis of coenzyme A (CoA) in bacteria and mammalian cells. It catalyzes the first committed step in the universal biosynthetic pathway leading to CoA and is itself subject to regulation through feedback inhibition by CoA. This family member is most abundant in muscle but is expressed in all tissues. [provided by RefSeq, Jul 2008]

Primary Disease Associations & Inheritance

?Cataract 49MIM #619593
AD
0
Active trials
5
Pubs (1 yr)
129
P/LP submissions
0%
P/LP missense
0.30
LOEUF· LoF intol.
Mechanism
Clinical SummaryPANK4
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.98). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
122 unique Pathogenic / Likely Pathogenic· 113 VUS of 262 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.30LOEUF
pLI 0.983
Z-score 4.89
OE 0.15 (0.080.30)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.42Z-score
OE missense 0.70 (0.640.76)
362 obs / 516.6 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.15 (0.080.30)
00.351.4
Missense OE0.70 (0.640.76)
00.61.4
Synonymous OE1.18
01.21.6
LoF obs/exp: 6 / 38.9Missense obs/exp: 362 / 516.6Syn Z: -2.13

ClinVar Variant Classifications

262 submitted variants in ClinVar

Classification Summary

Pathogenic120
Likely Pathogenic2
VUS113
Likely Benign8
Benign2
120
Pathogenic
2
Likely Pathogenic
113
VUS
8
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
120
0
120
Likely Pathogenic
0
0
2
0
2
VUS
0
96
17
0
113
Likely Benign
0
3
0
5
8
Benign
0
1
0
1
2
Total01001396245

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

PANK4 · 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 →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC