Glutamyl-/?-aminopropionitrile

The ingestion of diets containing certain peas of the genus Lathyrus may cause widespread lesions of connective tissue, referred to collectively as lathyrism -. The type of lesion varies from species to species but in the rat is characterized by skeletal deformities, neurological disturbances and blood vessel rupture. The active principle in the plant has been identified as i T-y-L-glutamyl-/3-aminopropionitrile. Similar effects can be obtained with /5-aminopropio-nitrile itself, whilst aminoacetonitrile is even more active. 

Among the amino acids from Lathyrus are 2,4-diamino-butyric acid (56) (a neurologically active factor) see above), A -oxalyl-2,3-diaminopropionic acid (57) (a potent neurotoxin), 3-cyanoalanine (58) (a neurotoxin), and the 4-gluta-myl derivative of 3-cyanoalanine. The decarboxylation product of 3-cyanoalanine, 3-aminopropionitrile (59), and its 4-glutamyl derivative induce many of the osteolathyritic skeletal abnormalities (Rosenthal, 1991) (Fig. 13.13). 

Consumption of seeds of the genus Lathyrus (Fabaceae) by man and his domestic animals produces a syndrome called lathyrism. Different amino acids produce the two forms of this malady neurolathyrism and osteolathyrism. 3-Aminopropionitrile 0-aminopropionitrile) (59) is the active osteolathyritic factor in Lathyrus species. Several factors are responsible for neurolathyrism. 3-Cyanoalanine (58), its 4-glutamyl derivative, 2,4-diaminobutyric acid (56), and the N-oxalyl derivative of 2,3-diaminopropionic (a,3-diamino-propionic) acid (57) are all known to be involved. These compounds are found primarily in plants of the genus iMth-yrus. 3-Cyanoalanine (58) and its 4-glutamyl derivative are also common in Vida species. Several related series of compounds are found in other members of the Fabaceae. 

Lathyrinogenic amino acids nonproteogenic amino acids occurring in the seeds of some species of vetch (Lathyrus). They include diaminobutyric acid H2N-(CH2)2-CH(NH2)- 00H (neurolathrinogenic effect), p-aminopropionitrile, which occurs as the glutamyl peptide in the seeds of Lathyrus. odoratus, and presumably the N-oxaloyl-a,p-diamino-propionic acid HOOC-CH(NH2)CH2-NH-CO-COOH. The disease caused in humans and animals by L.a.a. is called lathyrism, and takes various forms, e.g. neuro-(nerve)- and osteo(bone)-lathyrism e.g. PiAiy-gluta-rayI)aminopropionitrile causes skeletal abnormalities in rats... 

In Lathyrus species L- S-cyanoalanine is a precursor of other secondary products (Fig. 161). On the one hand by decarboxylation j -aminopropionitrile is formed and on the other hand by reduction diaminobutyric acid, an ornithine homolog, is built. Furthermore, in Leguminosae oxalyl and glutamyl derivatives of these amino acids occur. 

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