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Ataxia v2.203 SPG11 Bryony Thompson Marked gene: SPG11 as ready
Ataxia v2.203 SPG11 Bryony Thompson Gene: spg11 has been classified as Green List (High Evidence).
Ataxia v2.203 SPG11 Bryony Thompson Classified gene: SPG11 as Green List (high evidence)
Ataxia v2.203 SPG11 Bryony Thompson Gene: spg11 has been classified as Green List (High Evidence).
Ataxia v2.202 SPG11 Bryony Thompson gene: SPG11 was added
gene: SPG11 was added to Ataxia. Sources: Literature
Mode of inheritance for gene: SPG11 was set to BIALLELIC, autosomal or pseudoautosomal
Publications for gene: SPG11 were set to 40782215; 40730687; 39304850; 36530930; 35326432; 30778698; 29983107
Phenotypes for gene: SPG11 were set to hereditary spastic paraplegia 11, MONDO:0011445
Review for gene: SPG11 was set to GREEN
Added comment: Across seven studies, SPG11 has been implicated in 15 unrelated families (17 patients) with autosomal recessive hereditary spastic paraplegia type 11 presenting with mild cerebellar ataxia, spasticity, thin or absent corpus callosum and cognitive impairment; all families carry biallelic loss‑of‑function variants, confirming a loss‑of‑function disease mechanism.
Sources: Literature