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Osteolathyrism

Collagen cross-linking deficiency


Collagen cross-linking deficiency

FieldValue
nameOsteolathyrism
synonymsOdoratism, Lathyrism
fieldRheumatology
symptomsskeletal deformities, bone pain
durationPermanent
causesOver consumption of Lathyrus sativus
frequencyRare

Osteolathyrism, sometimes referred to as odoratism, is a form of the disease Lathyrism. The disease results from the ingestion of Lathyrus odoratus seeds (sweet peas). The toxin found in the sweet peas is (beta-aminopropionitrile), which affects the linking of collagen, a protein of connective tissues. The condition results in damage to bone and mesenchymal connective tissues.

Signs and symptoms

  • Bone pain
  • Skeletal deformity
  • Fatigue
  • Malnourishment

Cause

Aside from L. odoratus, other members of the genus are also known to cause the disease, including L. sylvestris, L. cicera, and L. clymenum. Other instigators are ureides, semicarbazides and thiosemicarbazides, which are believed to chelate the prosthetic Cu(II)-bipyridine cofactor complex in the enzyme lysyl oxidase.

Lysyl oxidase is an important enzyme for the creation of crosslinks between collagen triple-helices in connective tissue. By oxidizing the terminal amino group of lysine, an aldehyde is created. This aldehyde can undergo several reactions with neighboring aldehydes or amines to create strong covalent cross-links between collagen tertiary structures in bone and cartilage. The main product of these reactions is the aldimine compound dehydrohydroxylysinonorleucine. This unique crosslink can be formed by the Schiff base mechanism in which the lone pair of electrons on a primary amine react with the carbonyl carbon of an aldehyde. Other crosslinks include the formation of an α,β-unsaturated ketone via aldol condensation and hydroxylysinonorleucine.

If these crosslinks are not formed, as in the case of osteolathyrism, the synthesis of strong mesenchymal and mesodermal tissue is inhibited. Symptoms of osteolathyrism include weakness and fragility of connective tissue (i.e., skin, bones, and blood vessels (angiolathyrism) and the paralysis of the lower extremities associated with neurolathyrism. For these reasons, compounds containing lathyrogens should be avoided during pregnancy and growth of a child.

Prevention

Prevention of osteolathyrism can be achieved with a cessation of L. sativus consumption.

References

References

  1. (November 1954). "Incisor Ash Versus Femur Ash in Sweet Pea Lathyrism (Odoratism)". The Journal of Nutrition.
  2. Rosenthal, Gerald. (2003). "Plant Nonprotein Amino and Imino Acids: Biological, Biochemical, and Toxicological Properties". Elsevier.
  3. (May 2006). "Evidence of Osteolathyrism among patients suffering from Neurolathyrism in Bangladesh.". Natural Toxins.
  4. (2002). "Biochemical and toxicological evaluation of agent-cofactor reactivity as a mechanism of action for osteolathyrism". Toxicology.
  5. (1971). "The chemistry of the collagen cross-links. The absence of reduction of dehydrolysinonorleucine and dehydrohydroxylysinonorleucine in vivo". The Biochemical Journal.
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