MKS1

Chr 17AR

MKS transition zone complex subunit 1

Also known as: BBS13, JBTS28, MES, MKS, POC12

The protein encoded by this gene localizes to the basal body and is required for formation of the primary cilium in ciliated epithelial cells. Mutations in this gene result in Meckel syndrome type 1 and in Bardet-Biedl syndrome type 13. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2009]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Bardet-Biedl syndrome 13MIM #615990
AR
Joubert syndrome 28MIM #617121
AR
Meckel syndrome 1MIM #249000
AR
Meckel syndrome 1MIM #249000
AR

Clinical highlights

Gene-disease validity (ClinGen)
ciliopathy · ARDefinitivesufficient evidence for diagnostic panels
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.
5
Active trials
7
Pubs (1 yr)
P/LP submissions
P/LP missense
1.04
LOEUF
LOF
Mechanism· G2P
📖
GeneReview available — MKS1
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.04LOEUF
pLI 0.000
Z-score 1.35
OE 0.76 (0.571.04)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.49Z-score
OE missense 0.92 (0.841.02)
296 obs / 320.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.76 (0.571.04)
00.351.4
Missense OE?0.92 (0.841.02)
00.61.4
Synonymous OE?0.99
01.21.6
LoF obs/exp: 29 / 37.9Missense obs/exp: 296 / 320.8Syn Z: 0.06

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MKS1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.