Previous symbols: ALS4, SCAR1. Aliases: KIAA0625, AOA2, Sen1, STEX.
Relationship is the entry's genetic[].relationship_type. untyped means the entry has a genetic record for the gene but does not type it; its free-text association is shown instead.
| Disorder | Relationship | Where the gene appears |
|---|---|---|
| Ataxia With Oculomotor Apraxia Type 2 | causative germline | |
| Juvenile Amyotrophic Lateral Sclerosis | causative germline |
mechanism: Senataxin Helicase Dysfunction and RNA Processing Defect
subtype: ALS4
scoped to subtype: ALS4
|
An entry matches when ClinGen's MONDO disease is the entry's own disease term, a subtype term, or an exact-match mapping. An amber status marks an entry ClinGen supports the gene for (Definitive, Strong or Moderate) whose own record does not type it. A Limited, Disputed or Refuted tier on such an entry is not a gap.
| ClinGen disease | MOI | Classification | dismech entry |
|---|---|---|---|
| distal hereditary motor neuropathy MONDO:0018894 | AD | Definitive 2022-08-03 |
no dismech entry |
Identity from HGNC (snapshot 2026-10-01). Page layers are described in the gene pages documentation.