pqq cofactor and Menkes Kinky Hair Syndrome

pqq cofactor has been researched along with Menkes Kinky Hair Syndrome in 1 studies

PQQ Cofactor: A pyrrolo-quinoline having two adjacent keto-groups at the 4 and 5 positions and three acidic carboxyl groups. It is a coenzyme of some DEHYDROGENASES.
pyrroloquinoline quinone : A pyrroloquinoline having oxo groups at the 4- and 5-positions and carboxy groups at the 2-, 7- and 9-positions.

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)

Research

Studies (1)

Authors

Other Studies

1 other study available for pqq cofactor and Menkes Kinky Hair Syndrome