PAPSS2

Chr 10

3'-phosphoadenosine 5'-phosphosulfate synthase 2

Also known as: ATPSK2, BCYM4, SK2

Sulfation is a common modification of endogenous (lipids, proteins, and carbohydrates) and exogenous (xenobiotics and drugs) compounds. In mammals, the sulfate source is 3'-phosphoadenosine 5'-phosphosulfate (PAPS), created from ATP and inorganic sulfate. Two different tissue isoforms encoded by different genes synthesize PAPS. This gene encodes one of the two PAPS synthetases. Defects in this gene cause the Pakistani type of spondyloepimetaphyseal dysplasia. Two alternatively spliced transcript variants that encode different isoforms have been described for this gene. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtBrachyolmia type 4 with mild epiphyseal and metaphyseal changes

Clinical highlights

Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
0
Active trials
18
Pubs (1 yr)
P/LP submissions
P/LP missense
0.95
LOEUF
LOF
Mechanism· G2P
📖
GeneReview available — PAPSS2
Authoritative clinical overview · Recommended first read
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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?
0.95LOEUF
pLI 0.000
Z-score 1.75
OE 0.66 (0.460.95)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.46Z-score
OE missense 0.93 (0.851.02)
327 obs / 351.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.66 (0.460.95)
00.351.4
Missense OE?0.93 (0.851.02)
00.61.4
Synonymous OE?1.11
01.21.6
LoF obs/exp: 20 / 30.4Missense obs/exp: 327 / 351.3Syn Z: -0.96

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PAPSS2 · 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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