RGD Reference Report - Biosynthesis of heme in mammals. - Rat Genome Database

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Biosynthesis of heme in mammals.

Authors: Ajioka, RS  Phillips, JD  Kushner, JP 
Citation: Ajioka RS, etal., Biochim Biophys Acta. 2006 Jul;1763(7):723-36. Epub 2006 Jun 3.
RGD ID: 4144542
Pubmed: PMID:16839620   (View Abstract at PubMed)
DOI: DOI:10.1016/j.bbamcr.2006.05.005   (Journal Full-text)

Most iron in mammalian systems is routed to mitochondria to serve as a substrate for ferrochelatase. Ferrochelatase inserts iron into protoporphyrin IX to form heme which is incorporated into hemoglobin and cytochromes, the dominant hemoproteins in mammals. Tissue-specific regulatory features characterize the heme biosynthetic pathway. In erythroid cells, regulation is mediated by erythroid-specific transcription factors and the availability of iron as Fe/S clusters. In non-erythroid cells the pathway is regulated by heme-mediated feedback inhibition. All of the enzymes in the heme biosynthetic pathway have been crystallized and the crystal structures have permitted detailed analyses of enzyme mechanisms. All of the genes encoding the heme biosynthetic enzymes have been cloned and mutations of these genes are responsible for a group of human disorders designated the porphyrias and for X-linked sideroblastic anemia. The biochemistry, structural biology and the mechanisms of tissue-specific regulation are presented in this review along with the key features of the porphyric disorders.



Molecular Pathway Annotations    Click to see Annotation Detail View

RGD Manual Annotations


  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
ALADHumanheme biosynthetic pathway   TAS  RGD 
ALAS1Humanheme biosynthetic pathway   TAS  RGD 
ALAS2Humanheme biosynthetic pathway   TAS  RGD 
AladRatheme biosynthetic pathway   ISOALAD (Homo sapiens) RGD 
AladMouseheme biosynthetic pathway   ISOALAD (Homo sapiens) RGD 
Alas1Ratheme biosynthetic pathway   ISOALAS1 (Homo sapiens) RGD 
Alas1Mouseheme biosynthetic pathway   ISOALAS1 (Homo sapiens) RGD 
Alas2Ratheme biosynthetic pathway   ISOALAS2 (Homo sapiens) RGD 
Alas2Mouseheme biosynthetic pathway   ISOALAS2 (Homo sapiens) RGD 
CPOXHumanheme biosynthetic pathway   TAS  RGD 
CpoxRatheme biosynthetic pathway   ISOCPOX (Homo sapiens) RGD 
CpoxMouseheme biosynthetic pathway   ISOCPOX (Homo sapiens) RGD 
FECHHumanheme biosynthetic pathway   TAS  RGD 
FechRatheme biosynthetic pathway   ISOFECH (Homo sapiens) RGD 
FechMouseheme biosynthetic pathway   ISOFECH (Homo sapiens) RGD 
HMBSHumanheme biosynthetic pathway   TAS  RGD 
HmbsRatheme biosynthetic pathway   ISOHMBS (Homo sapiens) RGD 
HmbsMouseheme biosynthetic pathway   ISOHMBS (Homo sapiens) RGD 
PPOXHumanheme biosynthetic pathway   TAS  RGD 
PpoxRatheme biosynthetic pathway   ISOPPOX (Homo sapiens) RGD 
PpoxMouseheme biosynthetic pathway   ISOPPOX (Homo sapiens) RGD 
URODHumanheme biosynthetic pathway   TAS  RGD 
UROSHumanheme biosynthetic pathway   TAS  RGD 
UrodRatheme biosynthetic pathway   ISOUROD (Homo sapiens) RGD 
UrodMouseheme biosynthetic pathway   ISOUROD (Homo sapiens) RGD 
UrosRatheme biosynthetic pathway   ISOUROS (Homo sapiens) RGD 
UrosMouseheme biosynthetic pathway   ISOUROS (Homo sapiens) RGD 
Objects Annotated

Genes (Rattus norvegicus)
Alad  (aminolevulinate dehydratase)
Alas1  (5'-aminolevulinate synthase 1)
Alas2  (5'-aminolevulinate synthase 2)
Cpox  (coproporphyrinogen oxidase)
Fech  (ferrochelatase)
Hmbs  (hydroxymethylbilane synthase)
Ppox  (protoporphyrinogen oxidase)
Urod  (uroporphyrinogen decarboxylase)
Uros  (uroporphyrinogen III synthase)

Genes (Mus musculus)
Alad  (aminolevulinate, delta-, dehydratase)
Alas1  (aminolevulinic acid synthase 1)
Alas2  (aminolevulinic acid synthase 2, erythroid)
Cpox  (coproporphyrinogen oxidase)
Fech  (ferrochelatase)
Hmbs  (hydroxymethylbilane synthase)
Ppox  (protoporphyrinogen oxidase)
Urod  (uroporphyrinogen decarboxylase)
Uros  (uroporphyrinogen III synthase)

Genes (Homo sapiens)
ALAD  (aminolevulinate dehydratase)
ALAS1  (5'-aminolevulinate synthase 1)
ALAS2  (5'-aminolevulinate synthase 2)
CPOX  (coproporphyrinogen oxidase)
FECH  (ferrochelatase)
HMBS  (hydroxymethylbilane synthase)
PPOX  (protoporphyrinogen oxidase)
UROD  (uroporphyrinogen decarboxylase)
UROS  (uroporphyrinogen III synthase)


Additional Information