OPTN

Chr 10

optineurin

Also known as: ALS12, FIP2, GLC1E, HIP7, HYPL, NRP, TFIIIA-INTP

This gene encodes the coiled-coil containing protein optineurin. Optineurin may play a role in normal-tension glaucoma and adult-onset primary open angle glaucoma. Optineurin interacts with adenovirus E3-14.7K protein and may utilize tumor necrosis factor-alpha or Fas-ligand pathways to mediate apoptosis, inflammation or vasoconstriction. Optineurin may also function in cellular morphogenesis and membrane trafficking, vesicle trafficking, and transcription activation through its interactions with the RAB8, huntingtin, and transcription factor IIIA proteins. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtGlaucoma 1, open angle, E
UniProtGlaucoma, normal pressure
UniProtAmyotrophic lateral sclerosis 12 with or without frontotemporal dementia

Clinical highlights

Gene-disease validity (ClinGen)
amyotrophic lateral sclerosis type 12 · SDDefinitivesufficient evidence for diagnostic panels2 gene-disease associations curated in total
2
Active trials
314
Pubs (1 yr)
P/LP submissions
P/LP missense
1.26
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — OPTN
Authoritative clinical overview · Recommended first read
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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?
1.26LOEUF
pLI 0.000
Z-score 0.43
OE 0.92 (0.681.26)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.62Z-score
OE missense 0.90 (0.810.99)
267 obs / 297.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.92 (0.681.26)
00.351.4
Missense OE?0.90 (0.810.99)
00.61.4
Synonymous OE?1.07
01.21.6
LoF obs/exp: 28 / 30.6Missense obs/exp: 267 / 297.2Syn Z: -0.60

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

OPTN · protein map & ClinVar variants

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