NEFH

Chr 22

neurofilament heavy chain

Also known as: CMT2CC, NFH

Neurofilaments are type IV intermediate filament heteropolymers composed of light, medium, and heavy chains. Neurofilaments comprise the axoskeleton and functionally maintain neuronal caliber. They may also play a role in intracellular transport to axons and dendrites. This gene encodes the heavy neurofilament protein. This protein is commonly used as a biomarker of neuronal damage and susceptibility to amyotrophic lateral sclerosis (ALS) has been associated with mutations in this gene. [provided by RefSeq, Oct 2008]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtAmyotrophic lateral sclerosis
UniProtCharcot-Marie-Tooth disease, axonal, type 2CC

Clinical highlights

Gene-disease validity (ClinGen)
amyotrophic lateral sclerosis · ADLimitednot for standalone diagnostic reporting2 gene-disease associations curated in total
0
Active trials
35
Pubs (1 yr)
P/LP submissions
P/LP missense
1.06
LOEUF
Multiple*
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.06LOEUF
pLI 0.000
Z-score 1.28
OE 0.75 (0.531.06)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.52Z-score
OE missense 0.93 (0.871.01)
470 obs / 503.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.75 (0.531.06)
00.351.4
Missense OE?0.93 (0.871.01)
00.61.4
Synonymous OE?1.05
01.21.6
LoF obs/exp: 22 / 29.5Missense obs/exp: 470 / 503.0Syn Z: -0.52

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NEFH · 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 →