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protoporphyrin ix and Hepatolenticular Degeneration

protoporphyrin ix has been researched along with Hepatolenticular Degeneration in 1 studies

protoporphyrin IX: RN given refers to parent cpd; structure in Merck Index, 9th ed, #7685
protoporphyrin : A cyclic tetrapyrrole that consists of porphyrin bearing four methyl substituents at positions 3, 8, 13 and 17, two vinyl substituents at positions 7 and 12 and two 2-carboxyethyl substituents at positions 2 and 18. The parent of the class of protoporphyrins.

Hepatolenticular Degeneration: A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.

Research Excerpts

ExcerptRelevanceReference
"Cu2+ toxicity was examined in two hepatocellular carcinoma cell lines, HepG2 and Hep3B, with Hep3B cells containing an integrated hepatitis B virus genome."1.35Regulation of heme synthesis and proteasomal activity by copper: possible implications for Wilson's disease. ( Babushkin, T; Hait-Darshan, R; Malik, Z, 2009)

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (100.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Hait-Darshan, R1
Babushkin, T1
Malik, Z1

Other Studies

1 other study available for protoporphyrin ix and Hepatolenticular Degeneration

ArticleYear
Regulation of heme synthesis and proteasomal activity by copper: possible implications for Wilson's disease.
    Journal of environmental pathology, toxicology and oncology : official organ of the International Society for Environmental Toxicology and Cancer, 2009, Volume: 28, Issue:3

    Topics: Adenosine Triphosphatases; Carcinoma, Hepatocellular; Cation Transport Proteins; Cell Line, Tumor; C

2009