Shelf life (days): 1460.0
Formulation: A solid
Formal Name: 3-mercapto-D-valine-4,4,4-d3
Purity: ≥99% deuterated forms (d1-d3)
Formula Markup: C5H8D3NO2S
Formula Weight: 152.23056
CAS Number: 2925655-01-0
Notes: Penicillamine-d3 is intended for use as an internal standard for the quantification of D-penicillamine (Item No. 23955) by GC- or LC-MS. Penicillamine is an orally bioavailable copper chelator and penicillin degradation product.{40699,40700} It increases urinary and fecal copper excretion and decreases liver copper concentration in a rat model of copper overload when administered at 0.67 mmol/kg per day, but does not affect kidney, spleen, or brain copper levels.{40701} Penicillamine (100 mg/kg per day) dissolves copper-rich granules in hepatic lysosomes of Long-Evans cinnamon (LEC) rats, which spontaneously develop hepatic injury and acute hepatitis and have a mutation homologous to that of the human Wilson disease gene.{40702} Penicillamine has anticonvulsant and proconvulsant effects in mice when administered at 0.5 and 250 mg/kg, respectively, which are blocked by the nitric oxide synthase (NOS) inhibitors L-NAME (Item No. 80210) and 7-nitroindazole (Item No. 81340).{40703} Formulations containing penicillamine have been used to treat Wilson disease, cystinuria, and active rheumatoid arthritis.
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