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Hereditary spastic paraplegia linked to chromosome 14q11-q21: reduction of the SPG3 locus interval from 5.3 to 2.7 cM
  1. Shirley Rainiera,
  2. Peter Hederaa,
  3. David Alvaradoa,
  4. Xinping Zhaoa,
  5. Kleopas A Kleopab,
  6. Terry Heiman-Pattersonb,
  7. John K Finka,c
  1. aDepartment of Neurology, University of Michigan, Ann Arbor, Michigan, USA, bDepartment of Neurology, Hahneman University School of Medicine, Philadelphia PA, USA, cGeriatric Research Education Clinical Center, Ann Arbor Veteran's Affairs Medical Center, Ann Arbor, Michigan, USA
  1. Dr Fink, Room 5214 CCGCB, 1500 E Medical Center Drive, Ann Arbor, Michigan 48109-0940, USA, jkfink{at}

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Editor—Recently, Reid et al 1 reported reduction of the chromosome 12q locus for autosomal dominant hereditary spastic paraplegia. We now report reduction of the chromosome 14q locus for autosomal dominant hereditary spastic paraplegia (SPG3, OMIM 182600). Hereditary spastic paraplegia (HSP) (also known as Strümpell-Lorrain syndrome2) is a heterogeneous group of disorders characterised by progressive lower extremity weakness and spasticity. Gait disturbance may begin at any age and progresses insidiously. Postmortem studies show axonal degeneration that is maximal in the distal ends of the corticospinal tracts and fasciculus gracilis fibres3 4 (website

Loci for autosomal dominant HSP have been identified on chromosomes 2p22 (SPG4), 2q24-34 (SPG13), 8q23-q24 (SPG8), 12q13 (SPG10), 10q23.3-q24.2 (SPG9), 14q11-q21 (SPG3), 15q11.1 (SPG6), and 19q13 (SPG12).5-16 Forty-five percent of kindreds with autosomal dominant, uncomplicated HSP are linked to the SPG4 locus on chromosome 2p22. Mutations in a novel gene (designated …

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