GPANK1

Chr 6

G-patch domain and ankyrin repeats 1

Also known as: ANKRD59, BAT4, D6S54E, G5, GPATCH10

The GPANK1 protein is thought to function in immunity and is located within the major histocompatibility complex III region. Mutations cause autosomal recessive spastic paraplegia with intellectual disability, seizures, and progressive neurodegeneration typically beginning in early childhood. The gene is not highly constrained against loss-of-function variation.

OMIMResearchSummary from RefSeq
LOEUF 1.40
Clinical SummaryGPANK1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
9 unique Pathogenic / Likely Pathogenic· 72 VUS of 90 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.40LOEUF
pLI 0.000
Z-score 0.51
OE 0.85 (0.531.40)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.42Z-score
OE missense 0.92 (0.811.03)
189 obs / 206.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.85 (0.531.40)
00.351.4
Missense OE0.92 (0.811.03)
00.61.4
Synonymous OE0.84
01.21.6
LoF obs/exp: 11 / 13.0Missense obs/exp: 189 / 206.1Syn Z: 1.13

ClinVar Variant Classifications

90 submitted variants in ClinVar

Classification Summary

Pathogenic5
Likely Pathogenic4
VUS72
Likely Benign4
5
Pathogenic
4
Likely Pathogenic
72
VUS
4
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
5
0
5
Likely Pathogenic
0
0
4
0
4
VUS
0
68
4
0
72
Likely Benign
0
3
1
0
4
Benign
0
0
0
0
0
Total07114085

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

View in ClinVar →

Protein Context — Lollipop Plot

GPANK1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
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