penicillamine has been researched along with Menkes Kinky Hair Syndrome in 5 studies
Penicillamine: 3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.
penicillamine : An alpha-amino acid having the structure of valine substituted at the beta position with a sulfanyl group.
Menkes Kinky Hair Syndrome: An inherited disorder of copper metabolism transmitted as an X-linked trait and characterized by the infantile onset of HYPOTHERMIA, feeding difficulties, hypotonia, SEIZURES, bony deformities, pili torti (twisted hair), and severely impaired intellectual development. Defective copper transport across plasma and endoplasmic reticulum membranes results in copper being unavailable for the synthesis of several copper containing enzymes, including PROTEIN-LYSINE 6-OXIDASE; CERULOPLASMIN; and SUPEROXIDE DISMUTASE. Pathologic changes include defects in arterial elastin, neuronal loss, and gliosis. (From Menkes, Textbook of Child Neurology, 5th ed, p125)
| Excerpt | Relevance | Reference |
|---|---|---|
| "Excellent treatment options exist for Wilson disease, based on copper chelation." | 2.49 | Inborn errors of copper metabolism. ( Kaler, SG, 2013) |
| "Menkes' disease is a rare X-linked recessive inherited disorder of copper metabolism characterized by neurodegeneration, peculiar hair, and early death." | 1.27 | Menkes' disease: long-term treatment with copper and D-penicillamine. ( Baerlocher, K; Nadal, D, 1988) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 3 (60.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 2 (40.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Kaler, SG | 1 |
| Nomura, S | 1 |
| Nozaki, S | 1 |
| Hamazaki, T | 1 |
| Takeda, T | 1 |
| Ninomiya, E | 1 |
| Kudo, S | 1 |
| Hayashinaka, E | 1 |
| Wada, Y | 1 |
| Hiroki, T | 1 |
| Fujisawa, C | 1 |
| Kodama, H | 1 |
| Shintaku, H | 1 |
| Watanabe, Y | 1 |
| Prockop, DJ | 1 |
| Kivirikko, KI | 1 |
| Tuderman, L | 1 |
| Guzman, NA | 1 |
| Siegel, RC | 1 |
| Nadal, D | 1 |
| Baerlocher, K | 1 |
2 reviews available for penicillamine and Menkes Kinky Hair Syndrome
| Article | Year |
|---|---|
Inborn errors of copper metabolism.
Topics: Chelating Agents; Child; Copper; Cutis Laxa; Ehlers-Danlos Syndrome; Hepatolenticular Degeneration; | 2013 |
Lysyl oxidase.
Topics: Amino Acid Oxidoreductases; Aneurysm; Animals; Chickens; Collagen; Copper; Cutis Laxa; Dogs; Ehlers- | 1979 |
3 other studies available for penicillamine and Menkes Kinky Hair Syndrome
| Article | Year |
|---|---|
PET imaging analysis with 64Cu in disulfiram treatment for aberrant copper biodistribution in Menkes disease mouse model.
Topics: Animals; Autoradiography; Brain; Chelating Agents; Copper; Copper Radioisotopes; Disease Models, Ani | 2014 |
The biosynthesis of collagen and its disorders (second of two parts).
Topics: Aging; Animals; Colchicine; Collagen; Collagen Diseases; Ehlers-Danlos Syndrome; Glucosyltransferase | 1979 |
Menkes' disease: long-term treatment with copper and D-penicillamine.
Topics: Body Fluids; Brain; Brain Diseases, Metabolic; Copper; Drug Therapy, Combination; Electroencephalogr | 1988 |