RTTN

Chr 18

rotatin

Also known as: MSSP

This gene encodes a large protein whose specific function is unknown. Absence of the orthologous protein in mouse results in embryonic lethality with deficient axial rotation, abnormal differentiation of the neural tube, and randomized looping of the heart tube during development. In human, mutations in this gene are associated with polymicrogyria with seizures. In human fibroblasts this protein localizes at the ciliary basal bodies. Given the intracellular localization of this protein and the phenotypic effects of mutations, this gene is suspected of playing a role in the maintenance of normal ciliary structure which in turn effects the developmental process of left-right organ specification, axial rotation, and perhaps notochord development. [provided by RefSeq, Jan 2013]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMicrocephaly, short stature, and polymicrogyria with or without seizures

Clinical highlights

Gene-disease validity (ClinGen)
microcephalic primordial dwarfism due to RTTN deficiency · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
7
Pubs (1 yr)
P/LP submissions
P/LP missense
0.74
LOEUF
DN
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?
0.74LOEUF
pLI 0.000
Z-score 3.97
OE 0.61 (0.500.74)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.11Z-score
OE missense 0.99 (0.941.04)
1124 obs / 1134.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.61 (0.500.74)
00.351.4
Missense OE?0.99 (0.941.04)
00.61.4
Synonymous OE?1.03
01.21.6
LoF obs/exp: 73 / 120.0Missense obs/exp: 1124 / 1134.1Syn Z: -0.44

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RTTN · protein map & ClinVar variants

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