COL27A1

Chr 9AR

collagen type XXVII alpha 1 chain

Also known as: STLS

The protein is a fibrillar collagen that functions during cartilage calcification and the transition of cartilage to bone. Autosomal recessive mutations cause Steel syndrome, a skeletal dysplasia characterized by bone and cartilage abnormalities. The pathogenic mechanism involves disruption of normal bone and cartilage development due to defective collagen function.

Summary from RefSeq, OMIM, UniProt

Primary Disease Associations & Inheritance

Steel syndromeMIM #615155
AR
0
Active trials
12
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.31
LOEUF· LoF intol.
LOF
Mechanism· G2P
Clinical SummaryCOL27A1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.59) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.31LOEUF
pLI 0.586
Z-score 7.51
OE 0.22 (0.160.31)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
0.63Z-score
OE missense 0.95 (0.901.00)
1060 obs / 1119.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.22 (0.160.31)
00.351.4
Missense OE0.95 (0.901.00)
00.61.4
Synonymous OE1.07
01.21.6
LoF obs/exp: 24 / 108.3Missense obs/exp: 1060 / 1119.2Syn Z: -1.11

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

COL27A1 · 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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