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uroporphyrinogen iii and Porphyria, Acute Intermittent

uroporphyrinogen iii has been researched along with Porphyria, Acute Intermittent in 2 studies

Uroporphyrinogens: Porphyrinogens which are intermediates in heme biosynthesis. They have four acetic acid and four propionic acid side chains attached to the pyrrole rings. Uroporphyrinogen I and III are formed from polypyrryl methane in the presence of uroporphyrinogen III cosynthetase and uroporphyrin I synthetase, respectively. They can yield uroporphyrins by autooxidation or coproporphyrinogens by decarboxylation.

Porphyria, Acute Intermittent: An autosomal dominant porphyria that is due to a deficiency of HYDROXYMETHYLBILANE SYNTHASE in the LIVER, the third enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features are recurrent and life-threatening neurologic disturbances, ABDOMINAL PAIN, and elevated level of AMINOLEVULINIC ACID and PORPHOBILINOGEN in the urine.

Research Excerpts

ExcerptRelevanceReference
"We aimed to treat severe acute intermittent porphyria in a 19-year-old woman by liver transplantation."1.32Liver transplantation as a cure for acute intermittent porphyria. ( Badminton, MN; Bramhall, SR; Elder, GH; Elias, E; Orug, T; Rhodes, JM; Soonawalla, ZF, 2004)

Research

Studies (2)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's2 (100.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Soonawalla, ZF1
Orug, T1
Badminton, MN1
Elder, GH1
Rhodes, JM1
Bramhall, SR1
Elias, E1
Di Pierro, E1
Brancaleoni, V1
Cappellini, MD1

Other Studies

2 other studies available for uroporphyrinogen iii and Porphyria, Acute Intermittent

ArticleYear
Liver transplantation as a cure for acute intermittent porphyria.
    Lancet (London, England), 2004, Feb-28, Volume: 363, Issue:9410

    Topics: Adult; Aminolevulinic Acid; Female; Follow-Up Studies; Humans; Liver Transplantation; Porphyria, Acu

2004
Gene symbol: HMBS. Disease: Porphyria, acute intermittent.
    Human genetics, 2005, Volume: 116, Issue:6

    Topics: Base Sequence; Frameshift Mutation; Gene Deletion; Humans; Porphyria, Acute Intermittent; Uroporphyr

2005