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Hereditary ataxia, adult onset v9.10 ELOVL5 Ida Ertmanska changed review comment from: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. This variant has 2 alleles reported in gnomAD v4.1.1, no homozygotes. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected. No family genetic testing done.

PMID: 32314013 Gazulla et al., 2020
Family with 5 affected individuals with gait ataxia and intermittent diplopia. Vestibular impairment and sensory neuronopathy were also noted. WES demonstrated a heterozygous variant in ELOVL5, c.779A>G (p.Tyr260Cys), in four tested patients. Disease onset was during 5th and 6th decades. Variant is LIKELY BENIGN in ClinVar, reported in gnomAD v4.1.1 with MAF = 0.001286 (European Non-Finnish population), including 1 homozygote - way too common for a dominant disease.

PMID: 25065913 DiGregorio et al., 2014
Three SCA-affected Italian families (2 have confirmed shared ancestry, third may be more distantly related or same variant arose spontaneously). ELOVL5 heterozygous variant c.689G>T, p.Gly230Val cosegregated with disease. Variant is not present in gnomAD v4.1.1. Disease onset was around 34-45 years of age, with slowly progressive symptoms.
Another heterozygous missense variant, ELOVL5 c.214C>G, p.Leu72Val, was detected in a French family with SCA. Variant is reported in 1 heterozygote in gnomAD v4.1.1. The female proband had ataxia with age of onset 51 years.

FUNCTIONAL EVIDENCE:
PMID: 37199746 Ferrero et al., 2023 - same research group as PMID: 25065913
Functional evidence showing that ELOVL5 c.689G>T p.Gly230Val is proteotoxic - mouse cortical neurons were transduced with lentiviruses expressing GFP and an ELOVL5 protein (wild type or mutant). Wild-type ELOVL5 did not affect neuronal viability whereas expression of p.G230V decreased neuronal viability by 10%. ; to: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. This variant has 2 alleles reported in gnomAD v4.1.1, no homozygotes. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected. No family genetic testing done.

PMID: 32314013 Gazulla et al., 2020
Family with 5 affected individuals with gait ataxia and intermittent diplopia. Vestibular impairment and sensory neuronopathy were also noted. WES demonstrated a heterozygous variant in ELOVL5, c.779A>G (p.Tyr260Cys), in four tested patients. Disease onset was during 5th and 6th decades. Variant is LIKELY BENIGN in ClinVar, reported in gnomAD v4.1.1 with MAF = 0.001286 (European Non-Finnish population), including 1 homozygote - way too common for a dominant disease.

PMID: 25065913 DiGregorio et al., 2014
Three SCA-affected Italian families (2 have confirmed shared ancestry, third may be more distantly related or same variant arose spontaneously). ELOVL5 heterozygous variant c.689G>T, p.Gly230Val cosegregated with disease. Variant is not present in gnomAD v4.1.1. Disease onset was around 34-45 years of age, with slowly progressive symptoms.
Another heterozygous missense variant, ELOVL5 c.214C>G, p.Leu72Val, was detected in a French family with SCA. Variant is reported in 1 heterozygote in gnomAD v4.1.1. The female proband had ataxia with age of onset 51 years.

FUNCTIONAL EVIDENCE:
PMID: 37199746 Ferrero et al., 2023 - same research group as PMID: 25065913
Functional evidence showing that ELOVL5 c.689G>T p.Gly230Val is proteotoxic - mouse cortical neurons were transduced with lentiviruses expressing GFP and an ELOVL5 protein (wild type or mutant). Wild-type ELOVL5 did not affect neuronal viability whereas expression of p.G230V decreased neuronal viability by 10%.

This gene is associated with AD Spinocerebellar ataxia 38, MIM:615957 (OMIM accessed 14th Aug 2026).
Hereditary ataxia, adult onset v9.10 ELOVL5 Ida Ertmanska Phenotypes for gene: ELOVL5 were changed from Spinocerebellar ataxia 38, 615957; Spinocerebellar ataxia 36 615957 to Spinocerebellar ataxia 38, OMIM:615957; spinocerebellar ataxia type 38, MONDO:0014417
Hereditary ataxia, adult onset v9.9 ELOVL5 Ida Ertmanska Publications for gene: ELOVL5 were set to
Hereditary ataxia, adult onset v9.8 ELOVL5 Ida Ertmanska Mode of inheritance for gene: ELOVL5 was changed from MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Hereditary ataxia, adult onset v9.7 ELOVL5 Ida Ertmanska Mode of pathogenicity for gene: ELOVL5 was changed from Other - please provide details in the comments to Loss-of-function variants (as defined in pop up message) DO NOT cause this phenotype - please provide details in the comments
Hereditary ataxia, adult onset v9.6 ELOVL5 Ida Ertmanska Classified gene: ELOVL5 as Amber List (moderate evidence)
Hereditary ataxia, adult onset v9.6 ELOVL5 Ida Ertmanska Added comment: Comment on list classification: There are now 3 unrelated families reported in literature with 3 unique monoallelic ELOVL5 missense variants, affected by adult-onset spinocerebellar ataxia. There is also a fourth pedigree, reported in PMID: 32314013, with a likely benign variant with a high allele frequency in gnomAD (not counted). Based on available evidence, this gene can be promoted to Green at the next update.
Hereditary ataxia, adult onset v9.6 ELOVL5 Ida Ertmanska Gene: elovl5 has been classified as Amber List (Moderate Evidence).
Hereditary ataxia, adult onset v9.5 ELOVL5 Ida Ertmanska Tag Q3_26_promote_green tag was added to gene: ELOVL5.
Hereditary ataxia, adult onset v9.5 ELOVL5 Ida Ertmanska changed review comment from: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. This variant has 2 alleles reported in gnomAD v4.1.1, no homozygotes. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected. No family genetic testing done.

PMID: 32314013 Gazulla et al., 2020
Family with 5 affected individuals with gait ataxia and intermittent diplopia. Vestibular impairment and sensory neuronopathy were also noted. WES demonstrated a heterozygous variant in ELOVL5, c.779A>G (p.Tyr260Cys), in four tested patients. Disease onset was during 5th and 6th decades. Variant is LIKELY BENIGN in ClinVar, reported in gnomAD v4.1.1 with MAF = 0.001286 (European Non-Finnish population), including 1 homozygote - way too common for a dominant disease.

PMID: 25065913 DiGregorio et al., 2014
SCA-affected Italian family. ELOVL5 heterozygous variant c.689G>T, p.Gly230Val cosegregated with disease. Variant is not present in gnomAD v4.1.1.
Another heterozygous missense variant, ELOVL5 c.214C>G, p.Leu72Val, was detected in a French family with SCA. Variant is reported in 1 heterozygote in gnomAD v4.1.1.

FUNCTIONAL EVIDENCE:
PMID: 37199746 Ferrero et al., 2023 - same research group as PMID: 25065913
Functional evidence showing that ELOVL5 c.689G>T p.Gly230Val is proteotoxic - mouse cortical neurons were transduced with lentiviruses expressing GFP and an ELOVL5 protein (wild type or mutant). Wild-type ELOVL5 did not affect neuronal viability whereas expression of p.G230V decreased neuronal viability by 10%.; to: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. This variant has 2 alleles reported in gnomAD v4.1.1, no homozygotes. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected. No family genetic testing done.

PMID: 32314013 Gazulla et al., 2020
Family with 5 affected individuals with gait ataxia and intermittent diplopia. Vestibular impairment and sensory neuronopathy were also noted. WES demonstrated a heterozygous variant in ELOVL5, c.779A>G (p.Tyr260Cys), in four tested patients. Disease onset was during 5th and 6th decades. Variant is LIKELY BENIGN in ClinVar, reported in gnomAD v4.1.1 with MAF = 0.001286 (European Non-Finnish population), including 1 homozygote - way too common for a dominant disease.

PMID: 25065913 DiGregorio et al., 2014
Three SCA-affected Italian families (2 have confirmed shared ancestry, third may be more distantly related or same variant arose spontaneously). ELOVL5 heterozygous variant c.689G>T, p.Gly230Val cosegregated with disease. Variant is not present in gnomAD v4.1.1. Disease onset was around 34-45 years of age, with slowly progressive symptoms.
Another heterozygous missense variant, ELOVL5 c.214C>G, p.Leu72Val, was detected in a French family with SCA. Variant is reported in 1 heterozygote in gnomAD v4.1.1. The female proband had ataxia with age of onset 51 years.

FUNCTIONAL EVIDENCE:
PMID: 37199746 Ferrero et al., 2023 - same research group as PMID: 25065913
Functional evidence showing that ELOVL5 c.689G>T p.Gly230Val is proteotoxic - mouse cortical neurons were transduced with lentiviruses expressing GFP and an ELOVL5 protein (wild type or mutant). Wild-type ELOVL5 did not affect neuronal viability whereas expression of p.G230V decreased neuronal viability by 10%.
Hereditary ataxia, adult onset v9.5 ELOVL5 Ida Ertmanska changed review comment from: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected. No family genetic testing done.

PMID: 32314013 Gazulla et al., 2020
Family with 5 affected individuals with gait ataxia and intermittent diplopia. Vestibular impairment and sensory neuronopathy were also noted. WES demonstrated a heterozygous variant in ELOVL5, c.779A>G (p.Tyr260Cys), in four tested patients. Disease onset was during 5th and 6th decades. Variant is LIKELY BENIGN in ClinVar, reported in gnomAD v4.1.1 with MAF = 0.001286 (European Non-Finnish population), including 1 homozygote.

PMID: 25065913 DiGregorio et al., 2014
SCA-affected Italian family. ELOVL5 heterozygous variant c.689G>T, p.Gly230Val cosegregated with disease. Variant is not present in gnomAD v4.1.1.
Another heterozygous missense variant, ELOVL5 c.214C>G, p.Leu72Val, was detected in a French family with SCA. Variant is reported in 1 heterozygote in gnomAD v4.1.1.

FUNCTIONAL EVIDENCE:
PMID: 37199746 Ferrero et al., 2023 - same research group as PMID: 25065913
Functional evidence showing that ELOVL5 c.689G>T p.Gly230Val is proteotoxic - mouse cortical neurons were transduced with lentiviruses expressing GFP and an ELOVL5 protein (wild type or mutant). Wild-type ELOVL5 did not affect neuronal viability whereas expression of p.G230V decreased neuronal viability by 10%.; to: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. This variant has 2 alleles reported in gnomAD v4.1.1, no homozygotes. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected. No family genetic testing done.

PMID: 32314013 Gazulla et al., 2020
Family with 5 affected individuals with gait ataxia and intermittent diplopia. Vestibular impairment and sensory neuronopathy were also noted. WES demonstrated a heterozygous variant in ELOVL5, c.779A>G (p.Tyr260Cys), in four tested patients. Disease onset was during 5th and 6th decades. Variant is LIKELY BENIGN in ClinVar, reported in gnomAD v4.1.1 with MAF = 0.001286 (European Non-Finnish population), including 1 homozygote - way too common for a dominant disease.

PMID: 25065913 DiGregorio et al., 2014
SCA-affected Italian family. ELOVL5 heterozygous variant c.689G>T, p.Gly230Val cosegregated with disease. Variant is not present in gnomAD v4.1.1.
Another heterozygous missense variant, ELOVL5 c.214C>G, p.Leu72Val, was detected in a French family with SCA. Variant is reported in 1 heterozygote in gnomAD v4.1.1.

FUNCTIONAL EVIDENCE:
PMID: 37199746 Ferrero et al., 2023 - same research group as PMID: 25065913
Functional evidence showing that ELOVL5 c.689G>T p.Gly230Val is proteotoxic - mouse cortical neurons were transduced with lentiviruses expressing GFP and an ELOVL5 protein (wild type or mutant). Wild-type ELOVL5 did not affect neuronal viability whereas expression of p.G230V decreased neuronal viability by 10%.
Hereditary ataxia, adult onset v9.5 ELOVL5 Ida Ertmanska edited their review of gene: ELOVL5: Changed publications to: 25065913, 32314013, 37199746, 40940404
Hereditary ataxia, adult onset v9.5 ELOVL5 Ida Ertmanska changed review comment from: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected.; to: PMID: 40940404 Watanabe et al., 2025
Patient 3 - Japanese male, het for ELOVL5 c.179 C > T, p.S60F variant. Patient exhibited slowly progressive SCA with dysarthria, limb ataxia, and truncal ataxia, but no spasticity. Disease onset was at 37 years old; he became wheelchair dependent at 53 yrs. Brain stem and cerebellar atrophy noted. He had an additional feature of bladder and rectal disturbances. Family history: Mother, sibling, maternal uncle, and maternal aunt affected. No family genetic testing done.

PMID: 32314013 Gazulla et al., 2020
Family with 5 affected individuals with gait ataxia and intermittent diplopia. Vestibular impairment and sensory neuronopathy were also noted. WES demonstrated a heterozygous variant in ELOVL5, c.779A>G (p.Tyr260Cys), in four tested patients. Disease onset was during 5th and 6th decades. Variant is LIKELY BENIGN in ClinVar, reported in gnomAD v4.1.1 with MAF = 0.001286 (European Non-Finnish population), including 1 homozygote.

PMID: 25065913 DiGregorio et al., 2014
SCA-affected Italian family. ELOVL5 heterozygous variant c.689G>T, p.Gly230Val cosegregated with disease. Variant is not present in gnomAD v4.1.1.
Another heterozygous missense variant, ELOVL5 c.214C>G, p.Leu72Val, was detected in a French family with SCA. Variant is reported in 1 heterozygote in gnomAD v4.1.1.

FUNCTIONAL EVIDENCE:
PMID: 37199746 Ferrero et al., 2023 - same research group as PMID: 25065913
Functional evidence showing that ELOVL5 c.689G>T p.Gly230Val is proteotoxic - mouse cortical neurons were transduced with lentiviruses expressing GFP and an ELOVL5 protein (wild type or mutant). Wild-type ELOVL5 did not affect neuronal viability whereas expression of p.G230V decreased neuronal viability by 10%.
Hereditary ataxia, adult onset v9.5 ELOVL5 Ida Ertmanska reviewed gene: ELOVL5: Rating: GREEN; Mode of pathogenicity: None; Publications: 40940404; Phenotypes: Spinocerebellar ataxia 38, OMIM:615957, spinocerebellar ataxia type 38, MONDO:0014417; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Hereditary ataxia, adult onset v1.188 ELOVL5 Louise Daugherty commented on gene: ELOVL5: Downgraded rating from Green to Amber. As discussed with the GMS Neurology Specialist Test Group webex call 26th July 2019: The Specialist Test Group all agreed that there is only enough evidence to rate this gene Amber
Hereditary ataxia, adult onset v1.187 ELOVL5 Louise Daugherty Source Expert Review Amber was added to ELOVL5.
Rating Changed from Green List (high evidence) to Amber List (moderate evidence)
Hereditary ataxia, adult onset v1.16 ELOVL5 Louise Daugherty Classified gene: ELOVL5 as Green List (high evidence)
Hereditary ataxia, adult onset v1.16 ELOVL5 Louise Daugherty Gene: elovl5 has been classified as Green List (High Evidence).
Hereditary ataxia, adult onset v1.14 ELOVL5 Louise Daugherty commented on gene: ELOVL5: Review and rating submitted byJames Polke (North Bristol NHS Trust), on behalf of London North GLH for GMS Neurology specialist test group
Hereditary ataxia, adult onset v1.13 ELOVL5 Louise Daugherty Source London North GMS was added to ELOVL5.
Hereditary ataxia, adult onset v1.11 ELOVL5 James Polke reviewed gene: ELOVL5: Rating: RED; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance:
Hereditary ataxia, adult onset v1.9 ELOVL5 Louise Daugherty Added phenotypes Spinocerebellar ataxia 38, 615957 for gene: ELOVL5
Hereditary ataxia, adult onset v1.8 ELOVL5 Louise Daugherty reviewed gene: ELOVL5: Rating: AMBER; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance:
Hereditary ataxia, adult onset v1.7 ELOVL5 Tracy Lester reviewed gene: ELOVL5: Rating: GREEN; Mode of pathogenicity: Loss-of-function variants (as defined in pop up message) DO NOT cause this phenotype -please provide details in the comments; Publications: ; Phenotypes: Spinocerebellar ataxia 38, 615957; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted; Current diagnostic: yes
Hereditary ataxia, adult onset v1.2 ELOVL5 Louise Daugherty Source NHS GMS was added to ELOVL5.
Hereditary ataxia, adult onset v1.1 ELOVL5 Louise Daugherty Source Wessex and West Midlands GLH was added to ELOVL5.
Hereditary ataxia, adult onset v0.2 ELOVL5 Eleanor Williams gene: ELOVL5 was added
gene: ELOVL5 was added to Hereditary ataxia - adult onset. Sources: Hereditary ataxia v1.148,Expert Review Red
Mode of inheritance for gene: ELOVL5 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Phenotypes for gene: ELOVL5 were set to Spinocerebellar ataxia 36 615957
Mode of pathogenicity for gene: ELOVL5 was set to Other - please provide details in the comments