CHAMP1

Chr 13AD

chromosome alignment maintaining phosphoprotein 1

Also known as: C13orf8, CAMP, CHAMP, MRD40, NEDHILD, ZNF828

CHAMP1 encodes a zinc finger protein required for proper chromosome alignment at metaphase and accurate chromosome segregation during mitosis by maintaining spindle microtubule attachment to kinetochores. Mutations cause autosomal dominant neurodevelopmental disorder with hypotonia, impaired language, and dysmorphic features. This represents an autosomal dominant intellectual disability syndrome with early childhood onset affecting neurological development and growth.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismADLOEUF 0.271 OMIM phenotype
Clinical SummaryCHAMP1
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Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.99). One damaged copy is likely sufficient to cause disease.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.27LOEUF
pLI 0.992
Z-score 4.08
OE 0.09 (0.030.27)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
0.14Z-score
OE missense 0.98 (0.911.06)
420 obs / 428.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.09 (0.030.27)
00.351.4
Missense OE0.98 (0.911.06)
00.61.4
Synonymous OE1.15
01.21.6
LoF obs/exp: 2 / 23.2Missense obs/exp: 420 / 428.1Syn Z: -1.49
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveCHAMP1-related neurodevelopmental disorderLOFAD
DN
0.3097th %ile
GOF
0.13100th %ile
LOF
0.82top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · 1 literature citation · LOEUF 0.27

Literature Evidence

LOFUsing a combination of exome sequencing and array-based detection of chromosomal rearrangements, the Deciphering Developmental Disorders Study (2015) examined 1,133 children with severe undiagnosed developmental disorders and their parents. The authors identified 2 patients with neurodevelopmental dPMID:25533962

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CHAMP1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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