Spider lamb syndrome explained

Spider lamb syndrome, also known as spider syndrome[1] and more formally as ovine hereditary chondrodysplasia,[2] is a homozygous recessive disorder affecting the growth of cartilage and bone in sheep. The name derives from the limbs of afflicted animals being thin, elongated, and "spider-like".[3]

It is a semilethal trait,[4] which is thought to have been first observed in the 1970s,[5] and is most common in sheep of the Suffolk and Hampshire breeds.[6] These are both black-faced breeds of sheep; the syndrome has never been detected in white-faced breeds.[7]

The syndrome was an economically significant issue for sheep breeders in the 1980s, [8] but with strict testing and breeding programs it has become less common.[8]

The mutation which causes spider lamb syndrome is found on ovine chromosome 6,[9] and involves the inactivation of fibroblast growth factor receptor 3.[10] It has been compared to dwarfism in beef cattle.[7]

Afflicted animals may be visibly deformed at birth and unable to stand, or seemingly normal for the first 4 to 6 weeks of their lives.

Symptoms

Symptoms have been observed on fetal lambs as early as by the completion of the second gestational trimester.[11] Under normal production circumstances, the lambs usually do not survive past the neonatal period.[12] For this reason, the disease is considered semi-lethal. The disease typically affects the musculo-skeletal system. The clinical signs can include: skeletal abnormalities, twisted or humped spines, facial defects, bent legs, abnormally long legs, flat ribs, and underdeveloped muscles. Due to these symptoms, lambs cannot stand to nurse.

Spider lamb syndrome is untreatable, and in almost all cases, the lambs must be euthanized.[11]

Causes

Spider lamb syndrome is caused by a mutation to the gene for fibroblast growth factor receptor 3 (FGFR3), on ovine chromosome 6. FGFR3 is in the tyrosine kinase receptor family and its function is to restrict the proliferation of cartilage at the growth plates of the long bones: regulating ossification (the conversion of cartilage into bone), limiting skeletal elongation, and thereby ensuring that the limbs are the right length.

Notes and References

  1. http://www.sheepresearch.co.nz/publication_database/hereditary-chondrodysplasia-spider-syndrome-new-zealand-suffolk-lamb-american Hereditary chondrodysplasia ("spider syndrome") in a New Zealand Suffolk lamb of American origin.
  2. http://www.smallruminantresearch.com/article/0921-4488%2895%2900722-W/abstract Developmental progression of the Spider Lamb Syndrome
  3. 1698566 . 17033969 . 10.1086/508433 . 79 . 5 . A novel mutation in FGFR3 causes camptodactyly, tall stature, and hearing loss (CATSHL) syndrome . 2006 . Toydemir RM, Brassington AE, Bayrak-Toydemir P, Krakowiak PA, Jorde LB, Whitby FG, Longo N, Viskochil DH, Carey JC, Bamshad MJ . Am J Hum Genet . 935–41.
  4. http://www.vgl.ucdavis.edu/services/SpiderLamb.php Spider Lamb Syndrome: Introduction
  5. http://www.ag.ndsu.edu/archive/hettinge/livestock/Archive/spider98sdr.pdf Spider Lamb Syndrome - 1998 Sheep Day Report: The Test for Spider Lamb Syndrome Gene in Sheep
  6. http://www.merckvetmanual.com/mvm/index.jsp?cfile=htm/bc/90228.htm Spider Lamb Syndrome
  7. Web site: Spider Syndrome. Fitch. Gerald. Oklahoma Cooperative Extension Service. March 2017 .
  8. Web site: spider. ag.ansc.purdue.edu. 2019-03-28.
  9. http://www.udifmv.ulg.ac.be/genmol/Department/Publications/Cockett_1999.pdf Localization of the locus causing Spider Lamb Syndrome to the distal end of ovine Chromosome 6
  10. http://jas.fass.org/content/84/11/2942.full Enhanced skeletal growth of sheep heterozygous for an inactivated fibroblast growth factor receptor 3
  11. https://www.sciencedirect.com/science/article/pii/092144889500722W Developmental progression of the Spider Lamb Syndrome
  12. Beever. J. E.. Smit. M. A.. Meyers. S. N.. Hadfield. T. S.. Bottema. C.. Albretsen. J.. Cockett. N. E.. 2006. A single-base change in the tyrosine kinase II domain of ovine FGFR3 causes hereditary chondrodysplasia in sheep. Animal Genetics. en. 37. 1. 66–71. 10.1111/j.1365-2052.2005.01398.x. 16441300. 1365-2052.