PRTFDC1

Chr 10

phosphoribosyl transferase domain containing 1

Also known as: HHGP

The protein has minimal phosphoribosyltransferase activity and binds purine nucleotides but is not expected to significantly contribute to purine metabolism. Mutations cause intellectual disability with sparse hair and brachydactyly, inherited in an autosomal recessive pattern. The gene is not highly constrained against loss-of-function variants.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
7
Pubs (1 yr)
10
P/LP submissions
0%
P/LP missense
1.69
LOEUF
Mechanism
Clinical SummaryPRTFDC1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
10 unique Pathogenic / Likely Pathogenic· 41 VUS of 68 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.69LOEUF
pLI 0.000
Z-score -0.56
OE 1.15 (0.791.69)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.44Z-score
OE missense 0.89 (0.771.04)
115 obs / 128.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.15 (0.791.69)
00.351.4
Missense OE0.89 (0.771.04)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 18 / 15.6Missense obs/exp: 115 / 128.9Syn Z: 0.03

ClinVar Variant Classifications

68 submitted variants in ClinVar

Classification Summary

Pathogenic9
Likely Pathogenic1
VUS41
Likely Benign1
Benign2
9
Pathogenic
1
Likely Pathogenic
41
VUS
1
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
9
0
9
Likely Pathogenic
0
0
1
0
1
VUS
0
37
4
0
41
Likely Benign
0
1
0
0
1
Benign
0
0
1
1
2
Total03815154

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

View in ClinVar →

Protein Context — Lollipop Plot

PRTFDC1 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗