HEME BIOSYNTHETIC PATHWAY (PW:0000218)

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Description

Heme serves as a prosthetic group for enzymes and proteins involved in a broad range of important cellular processes such as oxygen transport and storage, photosynthesis, redox, signaling and drug metabolism. Iron-protoporphyrin IX, or heme b, is the parent molecule whose function depends on the associated polypeptide, and its modifications yield other forms of heme. Four pyrroles linked by a methine bridge form the planar tetrapyrrole unit of heme; each pyrrole coordinates the iron atom via a n

Pathway Diagram:

Elsevier Inc. coproporphyrinogen III ---> protoporphyrinogen Fe2+ heme b protoporphyrin IX protoporphyrinogen coproporphyrinogen III uroporphyrinogen III hydroxymethylbilane porphobilinogen 5-aminolevulinic acid coenzyme A glycine succinyl-CoA Alas1 Alas2 Alad Hmbs Uros Urod Cpox Tspo Ppox Fech coproporphyrinogen III ---> protoporphyrinogen Fe2+ ---> heme b protoporphyrin IX ---> heme b H+ Fech ---> H+ protoporphyrinogen ---> protoporphyrin IX hydroxymethylbilane ---> uroporphyrinogen III uroporphyrinogen III ---> coproporphyrinogen III 5-aminolevulinic acid ---> porphobilinogen porphobilinogen ---> hydroxymethylbilane glycine ---> 5-aminolevulinic acid succinyl-CoA ---> 5-aminolevulinic acid Slc25a38 Slc25a38 ---> 5-aminolevulinic acid Slc25a38 ---> glycine citric acid cycle pathway citric acid cycle pathway ---> succinyl-CoA CO2 glycine ---> CO2 succinyl-CoA ---> coenzyme A
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Genes in Pathway:

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heme biosynthetic pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Alad aminolevulinate dehydratase JBrowse link 5 78,368,867 78,379,206 RGD:1578396
RGD:11554188
RGD:4144542
G Alas1 5'-aminolevulinate synthase 1 JBrowse link 8 114,927,704 114,941,038 RGD:1578396
RGD:11554188
RGD:4144542
RGD:1601233
G Alas2 5'-aminolevulinate synthase 2 JBrowse link X 23,167,576 23,187,356 RGD:1578396
RGD:11554188
RGD:4144542
G Cpox coproporphyrinogen oxidase JBrowse link 11 44,039,665 44,049,648 RGD:1578396
RGD:11554188
RGD:4144542
G Fech ferrochelatase JBrowse link 18 59,941,992 59,975,192 RGD:1578396
RGD:11554188
RGD:4144542
G Hmbs hydroxymethylbilane synthase JBrowse link 8 48,667,278 48,674,673 RGD:1578396
RGD:11554188
RGD:4144542
RGD:2301704
G Ppox protoporphyrinogen oxidase JBrowse link 13 89,650,094 89,654,998 RGD:1578396
RGD:11554188
RGD:4144542
RGD:1599180
G Slc25a38 solute carrier family 25, member 38 JBrowse link 8 128,790,348 128,802,988 RGD:11554188
RGD:11556278
G Tspo translocator protein JBrowse link 7 124,460,358 124,470,610 RGD:11554188
G Urod uroporphyrinogen decarboxylase JBrowse link 5 135,855,429 135,859,515 RGD:1578396
RGD:11554188
RGD:4144542
RGD:2303399
G Uros uroporphyrinogen III synthase JBrowse link 1 205,755,766 205,778,170 RGD:1578396
RGD:11554188
RGD:4144823
RGD:4144542

Pathway Gene Annotations

Disease Annotations Associated with Genes in the heme biosynthetic pathway
Disease TermsGene Symbols
Abdominal PainHmbs
acute intermittent porphyriaHmbs , Ppox
Acute Intermittent Porphyria, Nonerythroid VariantHmbs
acute kidney failureAlad
acute porphyriaAlad , Cpox , Urod
adult respiratory distress syndromeAlad
ALAD-Deficiency PorphyriaAlad
anemiaAlad , Alas2
Arsenic PoisoningAlad
autosomal recessive pyridoxine-refractory sideroblastic anemia 2Slc25a38
bile duct diseaseFech
bilirubin metabolic disorderAlas2 , Hmbs
BlisterPpox
Breast NeoplasmsUrod
Charcot-Marie-Tooth disease dominant intermediate CUrod
Colorectal NeoplasmsPpox
congenital disorder of glycosylationHmbs
Congenital Disorder of Glycosylation Type 1JHmbs
CoproporphyriaCpox
cutaneous porphyriaAlas2 , Fech , Uros
Diabetes Mellitus, Experimental Alad , Alas1 , Hmbs
End Stage Liver DiseaseTspo
erythropoietic protoporphyriaAlas2 , Fech
Erythropoietic Protoporphyria, X-Linked DominantAlas2
Experimental ArthritisCpox
Experimental Liver CirrhosisTspo
Experimental Liver NeoplasmsAlas2
FatigueAlad
FibrosisFech
focal dermal hypoplasiaHmbs
genetic diseaseHmbs
Genetic Predisposition to DiseaseAlad
HarderoporphyriaCpox
Head and Neck NeoplasmsUrod
hematopoietic system diseaseAlad
hemochromatosisAlad , Alas2
hemolytic anemiaAlas2
hepatic encephalopathyTspo
Hepatic PorphyriasAlad , Cpox , Fech , Hmbs , Ppox , Urod
hepatoerythropoietic porphyriaUrod
hereditary coproporphyriaCpox
HypercholesterolemiaHmbs
hypertensionAlad
hypochromic anemiaAlas2 , Slc25a38
hypothyroidismAlad
JaundiceFech
kidney diseaseAlad
Lead PoisoningAlad
Lead Poisoning, Nervous SystemAlad
liver cirrhosisAlad
liver diseaseAlad , Cpox
Manganese PoisoningAlad
meningiomaAlad
migrainePpox
Multiple AbnormalitiesHmbs
neurohypophyseal diabetes insipidusPpox
porphyriaAlas1
porphyria cutanea tardaAlad , Cpox , Urod
Porphyria Cutanea Tarda, Type IUrod
Prostatic NeoplasmsAlad , Hmbs
pulmonary hypertensionAlas1
renal cell carcinomaAlad
RhabdomyolysisAlas1
seminomaHmbs
SepsisAlas1
sickle cell anemiaAlad , Hmbs
sideroblastic anemiaAlad , Alas2 , Slc25a38
sideroblastic anemia 1Alas2 , Slc25a38
status epilepticusAlad
urinary bladder cancerHmbs
variegate porphyriaPpox
visual epilepsyAlad
VomitingHmbs
X-linked sideroblastic anemia with ataxiaAlas2
Pathway Annotations Associated with Genes in the heme biosynthetic pathway

References Associated with the heme biosynthetic pathway:

Ontology Path Diagram:

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