Test Detail

SMA (Feline Spinal Muscular Atrophy)

Neurological · Cat

Spinal muscular atrophy (SMA) is a hereditary neurodegenerative disease of the Maine Coon characterized by progressive loss of motor neurons in the spinal cord. Without innervation, muscles atrophy gradually and symmetrically, especially in the limbs and trunk. Affected kittens begin weakness around 3-4 months and develop a progressive but disabling neuromuscular condition. There is no treatment; DNA testing prevents the production of affected litters.
Inheritance patternAutosomal recessive
Gene / MutationLIX1 (LNPEP), ~140 kb deletion associated with SMA of the Maine Coon (Fyfe et al. 2006, PMID 16899656). Multiplex LIX1 assay: WT 239+431 bp, mutant 342 bp. Genomic coordinates according to assembly (Fca126_mat1.0: g.159254616_159394886del) — verify with the laboratory.
PenetranceIn homozygotes the disease manifests clinically with variation in the rate of progression; heterozygotes are clinically normal. Formal penetrance figures: limited data (Fyfe et al. 2006, PMID 16899656).
Codeuytt
Turnaround time7 days
Price36,05 €

Incidence

Restricted to the Maine Coon. The carrier frequency described in breed surveys is moderate and variable by country and line (limited data). Systematic testing in breeding animals keeps cases at low levels in the populations that apply it.

Breeder management

- Test Maine Coon breeding animals before the first mating, along with the usual breeding panel of the breed
- Never cross carrier with carrier: 25% risk of affected kittens
- A carrier can be crossed with a clear individual, prioritizing clear offspring to eliminate the allele
- In kittens with progressive muscle weakness from 3-4 months, request neurological assessment and genetic testing
- Record the results alongside the pedigree and share them with the breed club

Specialist notes

The differential diagnosis includes hereditary feline myopathies, muscular dystrophy due to dystrophin deficiency (described in domestic short-haired cats and crosses), polyneuropathies (diabetic, deficiency-related) and myositis. Electromyography shows denervation and muscle biopsy shows grouped neurogenic atrophy; creatine kinase is normal or slightly elevated. Affected cats can live as companion animals with an adapted environment (ramps, low heights), although they must never be used in breeding.

References

1. Fyfe JC et al. 2006, deleción de ~140 kb asociada a la atrofia muscular espinal felina (PMID 16899656)
2. Wakeling EN et al. 2012, degeneración del motoneurona inferior en el modelo felino de SMA (PMID 22120001)
3. Kopke MA et al. 2022, miopatía miotubular ligada al X (MTM1) en un Maine coon: diagnóstico diferencial (PMID 35962713)
4. OMIA:001402 Atrofia muscular espinal felina

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