4735. l-Homocystine

Nomenclature

CAS number: 626-72-2; 462-10-2 (unspecified stereo)
(2S,2′S)-4,4′-Dithiobis[2-aminobutanoic acid]; l-4,4′-dithiobis[2-aminobutyric acid].
C8H16N2O4S2; mol wt 268.35.
C 35.81%, H 6.01%, N 10.44%, O 23.85%, S 23.90%.

Description and references

Naturally occurring, dimeric form of homocysteine, q.v. Appearance in urine is indicative of the metabolic disorder, cystathionine β-synthase deficiency. Prepn of dl-form from methionine: L. W. Butz, V. du Vigneaud, J. Biol. Chem. 99, 135 (1932). Synthesis by the “malonate” method: W. I. Patterson, V. du Vigneaud, ibid. 111, 393 (1935); from 3,6-bis(β-chloroethyl)-2,5-dioxopiperazine: H. R. Snyder, G. W. Cannon, J. Am. Chem. Soc. 66, 511 (1944) DOI. Prepn of d- and l-forms: V. du Vigneaud, W. I. Patterson, J. Biol. Chem. 109, 97 (1935); H. Miyazaki et al., Bull. Chem. Soc. Jpn. 66, 536 (1993) DOI. Asymmetric synthesis: M. Adamczyk et al., Tetrahedron: Asymmetry 10, 4151 (1999) DOI. Isotachophoretic determn in urine: N. Mizobuchi et al., J. Chromatogr. B 382, 321 (1986) DOI PubMed. LC-MS/MS determn in plasma: M. Tomaiuolo et al., ibid. 842, 64 (2006) DOI PubMed.

Chemical structure

Properties

Crystals, dec 281-284°. [α]D21 16° (H2O); [α]D26 +77° (1.0N HCl).

Derivative

dl-Form.

Nomenclature

CAS number: 870-93-9
Bis(γ-amino-γ-carboxypropyl)disulfide.

Properties

Platelets from water, dec 263-265°. pK1 1.59; pK2 2.54; pK3 8.52; pK4 9.44. Soly in water: 1 part per 5000.

Derivative

d-Form.

Nomenclature

CAS number: 6027-15-2

Properties

Crystals, dec 281-284°. [α]D21 +16° (H2O); [α]D26 77° (1.0N HCl).

Use

Biomarker for inherited cystathionine β-synthase deficiency.