GJC2

Chr 1ARAD

gap junction protein gamma 2

Also known as: CX46.6, Cx47, GJA12, HLD2, LMPH1C, LMPHM3, PMLDAR, SPG44

This gene encodes a gap junction protein. Gap junction proteins are members of a large family of homologous connexins and comprise 4 transmembrane, 2 extracellular, and 3 cytoplasmic domains. This gene plays a key role in central myelination and is involved in peripheral myelination in humans. Defects in this gene are the cause of autosomal recessive Pelizaeus-Merzbacher-like disease-1. [provided by RefSeq, Jul 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

?Spastic paraplegia 44, autosomal recessiveMIM #613206
AR
Leukodystrophy, hypomyelinating, 2MIM #608804
AR
Lymphatic malformation 3MIM #613480
AD

Clinical highlights

Gene-disease validity (ClinGen)
hypomyelinating leukodystrophy 2 · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
14
Pubs (1 yr)
P/LP submissions
P/LP missense
1.23
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — GJC2
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.23LOEUF
pLI 0.010
Z-score 1.17
OE 0.54 (0.261.23)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
1.87Z-score
OE missense 0.67 (0.590.76)
173 obs / 257.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.54 (0.261.23)
00.351.4
Missense OE?0.67 (0.590.76)
00.61.4
Synonymous OE?0.94
01.21.6
LoF obs/exp: 4 / 7.4Missense obs/exp: 173 / 257.5Syn Z: 0.52

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

GJC2 · 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 →