Reaction: ABCB6 transports porphyrin from cytosol to mitchondrial matrix
- in pathway: Mitochondrial ABC transporters
The human gene ABCB6 encodes a mitochondrial half-type ATP-binding cassette (ABC) protein MTABC3 which is uniquely located on the outer mitochondrial membrane and is functional as a homodimer (Krishnamurthy et al. 2006). It plays a crucial role in heme synthesis by mediating porphyrin uptake into mitochondria (Mitsuhashi et al. 2000, Krishnamurthy et al. 2006).
Reaction - small molecule participants:
Pi [cytosol]
ADP [cytosol]
porphyrin [mitochondrial matrix]
porphyrin [cytosol]
H2O [cytosol]
ATP [cytosol]
Reactome.org reaction link: R-HSA-1369065
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Reaction input - small molecules:
porphyrin
water
ATP(4-)
Reaction output - small molecules:
hydrogenphosphate
ADP(3-)
porphyrin
Reactome.org link: R-HSA-1369065