PGGT1B

Chr 5

protein geranylgeranyltransferase type I subunit beta

Also known as: BGGI, GGTI

Protein geranylgeranyltransferase type I (GGTase-I) transfers a geranylgeranyl group to the cysteine residue of candidate proteins containing a C-terminal CAAX motif in which 'A' is an aliphatic amino acid and 'X' is leucine (summarized by Zhang et al., 1994 [PubMed 8106351]). The enzyme is composed of a 48-kD alpha subunit (FNTA; MIM 134635) and a 43-kD beta subunit, encoded by the PGGT1B gene. The FNTA gene encodes the alpha subunit for both GGTase-I and the related enzyme farnesyltransferase.[supplied by OMIM, Mar 2010]

ResearchGenerating clinical summary…
GOFmechanismLOEUF 0.60
Clinical SummaryPGGT1B
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.31) despite low pLI — interpret in context.
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.60LOEUF
pLI 0.041
Z-score 2.84
OE 0.31 (0.170.60)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.01Z-score
OE missense 0.80 (0.700.91)
157 obs / 196.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.31 (0.170.60)
00.351.4
Missense OE?0.80 (0.700.91)
00.61.4
Synonymous OE?1.06
01.21.6
LoF obs/exp: 6 / 19.6Missense obs/exp: 157 / 196.8Syn Z: -0.39

This gene — mechanism propensity

DN
0.5181th %ile
GOF
0.76top 25%
LOF
0.3454th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PGGT1B · 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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