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Cosa (chi) è cystine - definizione
CHEMICAL COMPOUND
L-cystine; E921; L-Cystine; C6H12N2O4S2
Cystine
·noun A white crystalline substance, C3H7NSO2, containing sulphur, occuring as a constituent of certain rare urinary calculi, and occasionally found as a sediment in urine.
cystine
['s?sti:n, -t?n]
¦ noun Biochemistry an oxidized dimeric form of cysteine.
Origin
C19: from Gk kustis 'bladder' (because it was first isolated from urinary calculi) + -ine4.
Cystinosis
A LYSOSOMAL STORAGE DISEASE CHARACTERIZED BY THE ABNORMAL ACCUMULATION OF CYSTINE IN THE LYSOSOMES. IT FOLLOWS AN AUTOSOMAL RECESSIVE INHERITANCE PATTERN AND HAS MATERIAL BASIS IN MUTATIONS IN THE CTNS GENE, LOCATED ON CHROMOSOME 17.
Cystine diathesis; Cystine disease; Cystine storage disease; Cystinoses; Nephropathic cystinosis; Cystin transport, protein defect of; Cystinotic; Cistinosis
Cystinosis is a lysosomal storage disease characterized by the abnormal accumulation of cystine, the oxidized dimer of the amino acid cysteine. It is a genetic disorder that follows an autosomal recessive inheritance pattern.
Wikipedia
Cystine
Cystine is the oxidized derivative of the amino acid cysteine and has the formula (SCH2CH(NH2)CO2H)2. It is a white solid that is poorly soluble in water. As a residue in proteins, cystine serves two functions: a site of redox reactions and a mechanical linkage that allows proteins to retain their three-dimensional structure.
