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CMT IA

Autosomal RecessiveMitochondrialAutosomal DominantDe NovoVariableNeuropathy (Hereditary)OMIM 601097
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Overview

From WUSTL Neuromuscular

● PMP-22 Gene mutations Clinical-Gene relations Duplication Contiguous gene (YUHAL) Triplication Homozygous (Duplication) Point Allelic disorders Recessive CMT 1E (Deafness) Protein Pathology Also see: HNPP CMT 1APMP-22 Duplication Chaddock 1900 CMT 1A: Epidemiology Prevalence: 10.5 per 100,000 60% to 70% of Demyelinating CMT 40% to 50% of all CMT Genetics PMP-22 Gene mutation types Duplication of one PMP-22 gene (3 total copies of PMP-22): Types Segmental duplication in gene area Due to unequal crossing over of chromosomes during meiosis Duplication size Varies among patients Longer (Extended

OMIM Entries

OMIM #601097OMIM #611609OMIM #139393OMIM #600340OMIM #180800OMIM #118300OMIM #118220OMIM #616652OMIM #607642OMIM #610883

Related Conditions

Shared genes
Inclusion Body Myositis (IBM)
VAMPIADRB1
Myelin-related components, Other
TM

External Resources

WUSTL Neuromuscular
Washington University Disease Center
OMIM
Online Mendelian Inheritance in Man
PubMed
Biomedical Literature
GeneReviews
NCBI Expert-Authored Reviews
Orphanet
Portal for Rare Diseases
NORD
National Organization for Rare Disorders

Data sourced from the Washington University Neuromuscular Disease Center. For clinical use, always refer to primary sources.