MAP3K14

Chr 17AR

mitogen-activated protein kinase kinase kinase 14

Also known as: FTDCR1B, HS, HSNIK, IMD112, NIK

This gene encodes mitogen-activated protein kinase kinase kinase 14, which is a serine/threonine protein-kinase. This kinase binds to TRAF2 and stimulates NF-kappaB activity. It shares sequence similarity with several other MAPKK kinases. It participates in an NF-kappaB-inducing signalling cascade common to receptors of the tumour-necrosis/nerve-growth factor (TNF/NGF) family and to the interleukin-1 type-I receptor. [provided by RefSeq, Jul 2008]

Primary Disease Associations & Inheritance

Immunodeficiency 112MIM #620449
AR
0
Active trials
448
ClinVar variants
11
Pathogenic / LP
Missense Z
LOEUF
8
Pubs (2 yr)
Clinical SummaryMAP3K14
🧬
Gene-Disease Validity (ClinGen)
NIK deficiency · ARModerate

Moderate evidence — consider for supplementary testing

📋
ClinVar Variants
11 Pathogenic / Likely Pathogenic· 173 VUS of 448 total submissions
Some data sources returned errors (1)

ensembl: TimeoutError: The operation was aborted due to timeout

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

448 submitted variants in ClinVar

Classification Summary

Pathogenic11
VUS173
Likely Benign248
Benign12
Conflicting4
11
Pathogenic
173
VUS
248
Likely Benign
12
Benign
4
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
1
10
0
11
Likely Pathogenic
0
0
0
0
0
VUS
5
152
13
3
173
Likely Benign
1
9
74
164
248
Benign
0
4
7
1
12
Conflicting
4
Total6166104168448

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

MAP3K14 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

OMIM — Genotype-Phenotype Relationships

1 OMIM entry

Immunodeficiency 112

MIM #620449

Molecular basis of disorder known

Autosomal recessive
Clinical Literature
Landmark / reviewRecent case evidence
Key Publications
Landmark & review papers · by relevance
PubMed
MAP3K kinases and kidney injury.
Cuarental L et al.·Nefrologia (Engl Ed)
2019Review
Top 10 resultsSearch PubMed ↗

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →