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First, the cross-linkage of elastic and collagen fibers in the derms by lysyl oxidase was copper dependent.[sup][3] As D-penicillamine performed as a copper chelater, which indirectly inhibited the activity of lysyl oxidase, abnormal elastic fibers were formed and accumulated.
Thiol derivatives, such as tiopronin and D-penicillamine, cleave cystine into two cysteine moieties and combine with cysteine to form a highly soluble disulfide compound, decreasing the excretion of poorly solublefree cystine and increasing its solubility.
One of the most commonly used copper chelator is D-penicillamine. It is mainly used in acute intoxication.
Since 1955, d-penicillamine has been the most commonly used chelating agent9.
D-penicillamine (initial dose 10 mg/kg/day, maintenance dose 20 mg/kg/day, maximum dose 1 000-1 500 mg/day) and zinc (75 mg/day) treatment was given to all patients.
Ciprofloxacin (Java pharmaceutical, Pakistan), D-mannitol, D-penicillamine, Octanol, Stannous chloride, Gentistic acid (Sigma Aldrich, USA), Sodium pyrophosphate, Ascorbic acid, Acetone (Riedel, Germany), Saline (Otsuka, Pakistan), Tricholoacetic acid (Fisher Scientific, UK).
Complex (I) has been obtained by reaction of ferrous sulphate(II) with an aqueous solution of D-penicillamine in the molar ratio 1: 3.
D-penicillamine (D-pen), another copper-chelating agent, can generate intracellular reactive oxygen species (ROS) and be cytotoxic to human leukemia and breast cancer cells.
Some oral and topical agents including D-penicillamine has been used in some cases9 there are some therapeutic trials with dimethyl sulfoxide, ketotifen, and calcitriol.10 patients with JHF typically have severe physical limitations, but most individuals have normal intelligence and live into adulthood.
(3) Use of d-penicillamine resulted in some improvement in joint mobility secondary to its inhibitory effect on collagen maturation was documented in a few reports.
Animal data have shown decreased stricture formation with drugs that affect collagen deposition, including interferon-alfa-2b, octreotide, beta-aminopropionitrile (BAPN), N-acetylcysteine, and D-penicillamine [7, 8].
A single blind, randomized, parallel group clinical trial comparing the use of D-penicillamine with ECP was carried out in patients with SSc with progression of skin involvement during the past six months.