DOK7

Chr 4AR

docking protein 7

Also known as: C4orf25, CMS10, CMS1B

The protein encoded by this gene is essential for neuromuscular synaptogenesis. The protein functions in aneural activation of muscle-specific receptor kinase, which is required for postsynaptic differentiation, and in the subsequent clustering of the acetylcholine receptor in myotubes. This protein can also induce autophosphorylation of muscle-specific receptor kinase. Mutations in this gene are a cause of familial limb-girdle myasthenia autosomal recessive, which is also known as congenital myasthenic syndrome type 1B. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2009]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Fetal akinesia deformation sequence 3MIM #618389
AR
Myasthenic syndrome, congenital, 10MIM #254300
AR

Clinical highlights

Gene-disease validity (ClinGen)
congenital myasthenic syndrome 10 · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
29
Pubs (1 yr)
P/LP submissions
P/LP missense
0.93
LOEUF
Multiple*
Mechanism· predicted
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.93LOEUF
pLI 0.000
Z-score 1.77
OE 0.54 (0.320.93)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-2.55Z-score
OE missense 1.40 (1.301.51)
448 obs / 319.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.54 (0.320.93)
00.351.4
Missense OE?1.40 (1.301.51)
00.61.4
Synonymous OE?1.44
01.21.6
LoF obs/exp: 9 / 16.8Missense obs/exp: 448 / 319.8Syn Z: -4.35

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DOK7 · 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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