FOLH1B

Chr 11

folate hydrolase 1B (pseudogene)

Also known as: FOLH2, FOLHP, PSM, PSMA-LIKE, PSMAL

The protein functions as a metallocarboxypeptidase with folate hydrolase and N-acetylated-alpha-linked-acidic dipeptidase activities, involved in C-terminal protein deglutamylation. FOLH1B mutations cause autosomal recessive spastic paraplegia, presenting as progressive lower limb spasticity and weakness affecting the motor system.

OMIMResearchSummary from RefSeq, UniProt
DNmechanism
Clinical SummaryFOLH1B
📋
ClinVar Variants
17 unique Pathogenic / Likely Pathogenic· 8 VUS of 29 total submissions

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.7327th %ile
GOF
0.6151th %ile
LOF
0.2581th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

29 submitted variants in ClinVar

Classification Summary

Pathogenic16
Likely Pathogenic1
VUS8
Likely Benign4
16
Pathogenic
1
Likely Pathogenic
8
VUS
4
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
16
Likely Pathogenic
1
VUS
8
Likely Benign
4
Benign
0
Total29

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

FOLH1B · 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
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 2 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found