Biomaterial Database

[Congenital prophyria (Günther's disease)]. 1951

M DEROT, and J CANIVET, and P FALLOT

UI	MeSH Term	Description	Entries
D011161	Porifera	The phylum of sponges which are sessile, suspension-feeding, multicellular animals that utilize flagellated cells called choanocytes to circulate water. Most are hermaphroditic. They are probably an early evolutionary side branch that gave rise to no other group of animals. Except for about 150 freshwater species, sponges are marine animals. They are a source of ALKALOIDS; STEROLS; and other complex molecules useful in medicine and biological research.	Demospongiae,Sponges (Zoology),Sponge (Zoology),Sponges,Poriferas,Sponge
D011164	Porphyrias	A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.	Porphyria,Porphyrin Disorder,Disorder, Porphyrin,Disorders, Porphyrin,Porphyrin Disorders
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000818	Animals	Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA.	Animal,Metazoa,Animalia
D017092	Porphyria, Erythropoietic	An autosomal recessive porphyria that is due to a deficiency of UROPORPHYRINOGEN III SYNTHASE in the BONE MARROW; also known as congenital erythropoietic porphyria. This disease is characterized by SPLENOMEGALY; ANEMIA; photosensitivity; cutaneous lesions; accumulation of hydroxymethylbilane; and increased excretion of UROPORPHYRINS and COPROPORPHYRINS.	Deficiency of Uroporphyrinogen III Synthase,Erythropoietic Porphyria,Gunther's Disease,Porphyria, Erythropoietic, Congenital,Uroporphyrinogen III Synthase, Deficiency of,Congenital Erythropoietic Porphyria,Gunther Disease,Porphyria, Congenital Erythropoietic,UROS Deficiency,Congenital Erythropoietic Porphyrias,Erythropoietic Porphyria, Congenital,Erythropoietic Porphyrias,Erythropoietic Porphyrias, Congenital,Gunthers Disease,Porphyrias, Congenital Erythropoietic,Porphyrias, Erythropoietic