Names & Taxonomy

Uniprot ID:
Q9NP58
Entry Name:
ABCB6_HUMAN
Status:
reviewed
Protein Names:
ATP-binding cassette sub-family B member 6, mitochondrial (Mitochondrial ABC transporter 3) (Mt-ABC transporter 3) (P-glycoprotein-related protein) (Ubiquitously-expressed mammalian ABC half transporter)
Gene Names:
ABCB6 MTABC3 PRP UMAT
Gene Names Primary:
ABCB6
Organism:
Homo sapiens (Human)

Structure

Length:
842
Sequence:
MVTVGNYCEAEGPVGPAWMQDGLSPCFFFTLVPSTRMALGTLALVLALPCRRRERPAGADSLSWGAGPRISPYVLQLLLATLQAALPLAGLAGRVGTARGAPLPSYLLLASVLESLAGACGLWLLVVERSQARQRLAMGIWIKFRHSPGLLLLWTVAFAAENLALVSWNSPQWWWARADLGQQVQFSLWVLRYVVSGGLFVLGLWAPGLRPQSYTLQVHEEDQDVERSQVRSAAQQSTWRDFGRKLRLLSGYLWPRGSPALQLVVLICLGLMGLERALNVLVPIFYRNIVNLLTEKAPWNSLAWTVTSYVFLKFLQGGGTGSTGFVSNLRTFLWIRVQQFTSRRVELLIFSHLHELSLRWHLGRRTGEVLRIADRGTSSVTGLLSYLVFNVIPTLADIIIGIIYFSMFFNAWFGLIVFLCMSLYLTLTIVVTEWRTKFRRAMNTQENATRARAVDSLLNFETVKYYNAESYEVERYREAIIKYQGLEWKSSASLVLLNQTQNLVIGLGLLAGSLLCAYFVTEQKLQVGDYVLFGTYIIQLYMPLNWFGTYYRMIQTNFIDMENMFDLLKEETEVKDLPGAGPLRFQKGRIEFENVHFSYADGRETLQDVSFTVMPGQTLALVGPSGAGKSTILRLLFRFYDISSGCIRIDGQDISQVTQASLRSHIGVVPQDTVLFNDTIADNIRYGRVTAGNDEVEAAAQAAGIHDAIMAFPEGYRTQVGERGLKLSGGEKQRVAIARTILKAPGIILLDEATSALDTSNERAIQASLAKVCANRTTIVVAHRLSTVVNADQILVIKDGCIVERGRHEALLSRGGVYADMWQLQQGQEETSEDTKPQTMER
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Cell membrane

Function

Function:
Binds heme and porphyrins and functions in their ATP-dependent uptake into the mitochondria. Plays a crucial role in heme synthesis.
Gene Ontology Go:
ATP-binding cassette (ABC) transporter complex
endoplasmic reticulum
endoplasmic reticulum membrane
endosome
endosome membrane
extracellular exosome
Golgi apparatus
Golgi membrane
integral component of mitochondrial outer membrane
mitochondrial envelope
mitochondrial inner membrane
mitochondrial outer membrane
mitochondrion
plasma membrane
ATP binding
efflux transmembrane transporter activity
heme binding
heme transporter activity
heme-transporting ATPase activity
brain development
cellular iron ion homeostasis
heme transport
porphyrin-containing compound biosynthetic process
skin development
transmembrane transport
transport
Gene Ontology Biological Process:
brain development
cellular iron ion homeostasis
heme transport
porphyrin-containing compound biosynthetic process
skin development
transmembrane transport
transport
Gene Ontology Molecular Function:
ATP binding
efflux transmembrane transporter activity
heme binding
heme transporter activity
heme-transporting ATPase activity
Gene Ontology Cellular Component:
ATP-binding cassette (ABC) transporter complex
endoplasmic reticulum
endoplasmic reticulum membrane
endosome
endosome membrane
extracellular exosome
Golgi apparatus
Golgi membrane
integral component of mitochondrial outer membrane
mitochondrial envelope
mitochondrial inner membrane
mitochondrial outer membrane
mitochondrion
plasma membrane
Keywords:
3D-structure
ATP-binding
Alternative splicing
Cell membrane
Complete proteome
Disease mutation
Dyskeratosis congenita
Endoplasmic reticulum
Endosome
Golgi apparatus
Membrane
Microphthalmia
Mitochondrion
Mitochondrion outer membrane
Nucleotide-binding
Polymorphism
Reference proteome
Transmembrane
Transmembrane helix
Transport

Publication

PubMed ID:
10837493 11955620 14702039 15489334 17006453 17661442 18279659 22226084 22246506 23180570 24275569 16791740 20823549 16959974 23519333 24224009 25288164 24498303