disulfiram has been researched along with Menkes Kinky Hair Syndrome in 4 studies
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 |
|---|---|---|
| "Disulfiram was orally given, in addition to a parenteral administration of copper-histidine in the case of MD patients." | 2.77 | Effects of disulfiram treatment in patients with Menkes disease and occipital horn syndrome. ( Kodama, H; Ogawa, E, 2012) |
| "Pretreatment of disulfiram in MD model mice increased the copper level in the brain (0." | 1.48 | Disulfiram enhanced delivery of orally administered copper into the central nervous system in Menkes disease mouse model. ( Hamazaki, T; Hoshina, T; Kodama, H; Kudo, S; Nakatani, Y; Nozaki, S; Shintaku, H; Watanabe, Y, 2018) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 4 (100.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Hoshina, T | 1 |
| Nozaki, S | 2 |
| Hamazaki, T | 2 |
| Kudo, S | 2 |
| Nakatani, Y | 1 |
| Kodama, H | 4 |
| Shintaku, H | 2 |
| Watanabe, Y | 2 |
| Nomura, S | 1 |
| Takeda, T | 1 |
| Ninomiya, E | 1 |
| Hayashinaka, E | 1 |
| Wada, Y | 1 |
| Hiroki, T | 2 |
| Fujisawa, C | 2 |
| Ogawa, E | 2 |
| Bhadhprasit, W | 1 |
1 trial available for disulfiram and Menkes Kinky Hair Syndrome
| Article | Year |
|---|---|
Effects of disulfiram treatment in patients with Menkes disease and occipital horn syndrome.
Topics: Adolescent; Adult; Ceruloplasmin; Copper; Cutis Laxa; Disulfiram; Dopamine; Ehlers-Danlos Syndrome; | 2012 |
3 other studies available for disulfiram and Menkes Kinky Hair Syndrome
| Article | Year |
|---|---|
Disulfiram enhanced delivery of orally administered copper into the central nervous system in Menkes disease mouse model.
Topics: Animals; Autoradiography; Blood-Brain Barrier; Copper; Disease Models, Animal; Disulfiram; Drug Carr | 2018 |
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 |
Effect of copper and disulfiram combination therapy on the macular mouse, a model of Menkes disease.
Topics: Animals; Blood-Brain Barrier; Copper; Disease Models, Animal; Disulfiram; Electron Transport Complex | 2012 |