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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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PMP2
peripheral myelin protein 2
Chromosome 8 Β· 8q21.13
NCBI Gene: 5375Ensembl: ENSG00000147588.7HGNC: HGNC:9117UniProt: E5RH45
35PubMed Papers
21Diseases
0Drugs
6Pathogenic Variants
FUNCTIONAL ROLE
Transporter
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein bindingfatty acid bindingextracellular exosomecholesterol bindingCharcot-Marie-Tooth disease type 1Gperipheral neuropathygenetic disorderCharcot-Marie-Tooth disease
✦AI Summary

PMP2 (peripheral myelin protein 2) is a small, basic cytoplasmic protein that functions as a lipid-binding protein in peripheral myelin 1. The protein localizes to the cytoplasm and may play a role in lipid transport within Schwann cells, with potential cholesterol binding capacity. The human PMP2 gene spans approximately 8 kb on chromosome 8.3-q22.1 and consists of four exons with a TATA-like box in its 5'-flanking region 1. PMP2 is clinically significant as mutations in this gene cause Charcot-Marie-Tooth disease type 1G (CMT1G), a rare autosomal dominant demyelinating peripheral neuropathy 2. Affected patients typically present with childhood-onset distal muscle weakness, hypoesthesia, foot deformity, and areflexia, with slow progression predominantly affecting lower limbs 2. The protein appears to be part of myelination gene programs, as evidenced by its involvement in glial differentiation pathways 3. Beyond peripheral neuropathy, PMP2 expression changes have been observed in brain disorders, with reduced expression noted in suicide cases and potential diagnostic relevance in Alzheimer's disease through extracellular vesicles 45. However, pathogenic PMP2 variants appear to be rare in some populations, including Chinese CMT patients 6.

Sources cited
1
PMP2 structure, chromosomal location, and basic protein characteristics
PMID: 8288226
2
Clinical features of CMT1G caused by PMP2 mutations and disease phenotype
PMID: 37238449
3
PMP2 involvement in myelination gene programs
PMID: 37436963
4
Reduced PMP2 expression in suicide cases
PMID: 34923210
5
PMP2 in extracellular vesicles and potential Alzheimer's diagnostic relevance
PMID: 40168986
6
Rarity of pathogenic PMP2 variants in Chinese CMT patients
PMID: 29336362
Disease Associationsβ“˜21
Charcot-Marie-Tooth disease type 1GOpen Targets
0.71Strong
peripheral neuropathyOpen Targets
0.34Weak
genetic disorderOpen Targets
0.33Weak
Charcot-Marie-Tooth diseaseOpen Targets
0.19Weak
Charcot-Marie-Tooth disease type 1EOpen Targets
0.12Weak
hereditary neuropathy with liability to pressure palsiesOpen Targets
0.04Suggestive
Charcot-Marie-Tooth disease type 2B2Open Targets
0.03Suggestive
Barrett's esophagusOpen Targets
0.03Suggestive
medulloblastomaOpen Targets
0.03Suggestive
atypical teratoid rhabdoid tumorOpen Targets
0.02Suggestive
Alzheimer diseaseOpen Targets
0.02Suggestive
AnisometropiaOpen Targets
0.02Suggestive
colorectal adenocarcinomaOpen Targets
0.02Suggestive
nephrotic syndromeOpen Targets
0.02Suggestive
cancerOpen Targets
0.02Suggestive
large cell medulloblastomaOpen Targets
0.02Suggestive
melanomaOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.02Suggestive
Charcot-Marie-Tooth disease type 1Open Targets
0.01Suggestive
posterior cortical atrophyOpen Targets
0.01Suggestive
Charcot-Marie-Tooth disease, demyelinating, type 1GUniProt
Pathogenic Variants6
NM_002677.5(PMP2):c.144TAT[1] (p.Ile50del)Pathogenic
not provided|Peripheral neuropathy|Charcot-Marie-Tooth disease, demyelinating, type 1G
β˜…β˜…β˜†β˜†2025β†’ Residue 50
NM_002677.5(PMP2):c.155T>C (p.Ile52Thr)Pathogenic
Charcot-Marie-Tooth disease, demyelinating, type 1G|Inborn genetic diseases|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 52
NM_002677.5(PMP2):c.128T>A (p.Ile43Asn)Pathogenic
Peripheral neuropathy|Charcot-Marie-Tooth disease, demyelinating, type 1G|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 43
NM_002677.5(PMP2):c.167G>T (p.Ser56Ile)Likely pathogenic
Charcot-Marie-Tooth disease, demyelinating, type 1G
β˜…β˜†β˜†β˜†2024β†’ Residue 56
NM_002677.5(PMP2):c.151A>C (p.Thr51Pro)Pathogenic
Charcot-Marie-Tooth disease, demyelinating, type 1G
β˜†β˜†β˜†β˜†2025β†’ Residue 51
NM_002677.5(PMP2):c.128T>G (p.Ile43Ser)Likely pathogenic
Charcot-Marie-Tooth disease, demyelinating, type 1G
β˜†β˜†β˜†β˜†β†’ Residue 43
View on ClinVar β†—
Related Genes
RNMTProtein interaction88%PRXProtein interaction87%GDAP1Protein interaction84%HSCBProtein interaction75%CDH17Protein interaction75%CDH1Protein interaction70%
Tissue Expression6 tissues
Brain
100%
Heart
4%
Liver
0%
Ovary
0%
Bone Marrow
0%
Lung
0%
Gene Interaction Network
Click a node to explore
PMP2RNMTPRXGDAP1HSCBCDH17CDH1
PROTEIN STRUCTURE
Preparing viewer…
PDB6S2M Β· 0.72 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.51LoF Tolerant
pLIβ“˜
0.01Tolerant
Observed/Expected LoF0.78 [0.42–1.51]
RankingsWhere PMP2 stands among ~20K protein-coding genes
  • #11,038of 20,598
    Most Researched35
  • #3,420of 5,498
    Most Pathogenic Variants6
  • #15,226of 17,882
    Most Constrained (LOEUF)1.51
Genes detectedPMP2
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Molecular pathways and diagnosis in spatially resolved Alzheimer's hippocampal atlas.
PMID: 40168986
Neuron Β· 2025
1.00
2
A 20-year Clinical and Genetic Neuromuscular Cohort Analysis in Lebanon: An International Effort.
PMID: 34602496
J Neuromuscul Dis Β· 2022
0.90
3
Structure and localization of the gene encoding human peripheral myelin protein 2 (PMP2).
PMID: 8288226
Genomics Β· 1993
0.80
4
A Novel Family with Demyelinating Charcot-Marie-Tooth Disease Caused by a Mutation in the PMP2 Gene: A Case Series of Nine Patients and a Brief Review of the Literature.
PMID: 37238449
Children (Basel) Β· 2023
0.70
5
A review and meta-analysis of gene expression profiles in suicide.
PMID: 34923210
Eur Neuropsychopharmacol Β· 2022
0.60