Abstract
Hereditary canine spinal muscular atrophy (HCSMA), a dominantly inherited disorder of motor neurons, has three phenotypes: accelerated, intermediate, and chronic. In the accelerated and intermediate phenotypes, axonal sizes in ventral roots were smaller than in controls. Reductions in axonal size occurred primarily in large axons, and the frequency of small-caliber axons was increased. In HCSMA, nerve fiber shape, i.e., circularity, was reduced, and the relative thickness of the myelin sheath as a function of axonal caliber was decreased. The density of fibers in motor nerves was increased, making it unlikely that a selective loss of large-caliber axons explained the increased frequency of small-caliber axons. These observations suggest that, in HCSMA, changes in axonal size in motor nerves are associated with both growth arrest and axonal atrophy.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 333-342 |
| Number of pages | 10 |
| Journal | Laboratory Investigation |
| Volume | 61 |
| Issue number | 3 |
| State | Published - 1989 |
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ASJC Scopus subject areas
- Pathology and Forensic Medicine
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Changes in size of motor axons in hereditary canine spinal muscular atrophy. / Cork, L. C.; Struble, R. G.; Gold, B. G.; DiCarlo, C.; Fahnestock, K. E.; Griffin, J. W.; Price, D. L.
In: Laboratory Investigation, Vol. 61, No. 3, 1989, p. 333-342.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Changes in size of motor axons in hereditary canine spinal muscular atrophy
AU - Cork, L. C.
AU - Struble, R. G.
AU - Gold, B. G.
AU - DiCarlo, C.
AU - Fahnestock, K. E.
AU - Griffin, J. W.
AU - Price, D. L.
PY - 1989
Y1 - 1989
N2 - Hereditary canine spinal muscular atrophy (HCSMA), a dominantly inherited disorder of motor neurons, has three phenotypes: accelerated, intermediate, and chronic. In the accelerated and intermediate phenotypes, axonal sizes in ventral roots were smaller than in controls. Reductions in axonal size occurred primarily in large axons, and the frequency of small-caliber axons was increased. In HCSMA, nerve fiber shape, i.e., circularity, was reduced, and the relative thickness of the myelin sheath as a function of axonal caliber was decreased. The density of fibers in motor nerves was increased, making it unlikely that a selective loss of large-caliber axons explained the increased frequency of small-caliber axons. These observations suggest that, in HCSMA, changes in axonal size in motor nerves are associated with both growth arrest and axonal atrophy.
AB - Hereditary canine spinal muscular atrophy (HCSMA), a dominantly inherited disorder of motor neurons, has three phenotypes: accelerated, intermediate, and chronic. In the accelerated and intermediate phenotypes, axonal sizes in ventral roots were smaller than in controls. Reductions in axonal size occurred primarily in large axons, and the frequency of small-caliber axons was increased. In HCSMA, nerve fiber shape, i.e., circularity, was reduced, and the relative thickness of the myelin sheath as a function of axonal caliber was decreased. The density of fibers in motor nerves was increased, making it unlikely that a selective loss of large-caliber axons explained the increased frequency of small-caliber axons. These observations suggest that, in HCSMA, changes in axonal size in motor nerves are associated with both growth arrest and axonal atrophy.
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UR - http://www.scopus.com/inward/citedby.url?scp=0024460664&partnerID=8YFLogxK
M3 - Article
C2 - 2770248
AN - SCOPUS:0024460664
VL - 61
SP - 333
EP - 342
JO - Laboratory Investigation
JF - Laboratory Investigation
SN - 0023-6837
IS - 3
ER -