GLB1L2

Chr 11

galactosidase beta 1 like 2

Also known as: MST114, MSTP114

The protein cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. This gene is not well-constrained against loss-of-function variants (LOEUF 1.12), and no established human diseases have been definitively linked to GLB1L2 mutations based on current evidence. Further research is needed to establish clear genotype-phenotype correlations for this gene.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
88
P/LP submissions
0%
P/LP missense
1.12
LOEUF
Mechanism
Clinical SummaryGLB1L2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
88 unique Pathogenic / Likely Pathogenic· 115 VUS of 244 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.12LOEUF
pLI 0.000
Z-score 0.95
OE 0.83 (0.631.12)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.17Z-score
OE missense 1.02 (0.941.12)
379 obs / 369.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.83 (0.631.12)
00.351.4
Missense OE1.02 (0.941.12)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 32 / 38.4Missense obs/exp: 379 / 369.8Syn Z: 0.59

ClinVar Variant Classifications

244 submitted variants in ClinVar

Classification Summary

Pathogenic81
Likely Pathogenic7
VUS115
Likely Benign8
Benign3
81
Pathogenic
7
Likely Pathogenic
115
VUS
8
Likely Benign
3
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
81
0
81
Likely Pathogenic
0
0
7
0
7
VUS
0
105
10
0
115
Likely Benign
0
5
3
0
8
Benign
0
1
2
0
3
Total01111030214

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

View in ClinVar →

Protein Context — Lollipop Plot

GLB1L2 · 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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Clinical Literature
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