KEGG   PATHWAY: hsa02010Help
Entry
hsa02010                    Pathway                                

Name
ABC transporters - Homo sapiens (human)
Description
The ATP-binding cassette (ABC) transporters form one of the largest known protein families, and are widespread in bacteria, archaea, and eukaryotes. They couple ATP hydrolysis to active transport of a wide variety of substrates such as ions, sugars, lipids, sterols, peptides, proteins, and drugs. The structure of a prokaryotic ABC transporter usually consists of three components; typically two integral membrane proteins each having six transmembrane segments, two peripheral proteins that bind and hydrolyze ATP, and a periplasmic (or lipoprotein) substrate-binding protein. Many of the genes for the three components form operons as in fact observed in many bacterial and archaeal genomes. On the other hand, in a typical eukaryotic ABC transporter, the membrane spanning protein and the ATP-binding protein are fused, forming a multi-domain protein with the membrane-spanning domain (MSD) and the nucleotide-binding domain (NBD).
Class
Environmental Information Processing; Membrane transport
BRITE hierarchy
Pathway map
ABC transporters
hsa02010

All organismsOrtholog table
Disease
H00152  
Sitosterolemia
H00159  
Tangier disease
H00176  
Adrenoleukodystrophy (ALD)
H00208  
Hyperbilirubinemia
H00217  
Pulmonary surfactant metabolism dysfunction (SMDP)
H00218  
Cystic fibrosis (CF)
H00560  
Pseudoxanthoma elasticum
H00624  
Familial cholestasis
H00733  
Harlequin ichthyosis
H00734  
Lamellar ichthyosis (LI) and Non-bullous congenital ichthyosiform erythroderma (NBCIE)
H00819  
Stargardt disease (STGD)
H00821  
Macular degeneration
H00930  
Hypoalphalipoproteinemia
H00984  
Bare lymphocyte syndrome (BLS) type1
H01033  
Congenital bilateral absence of vas deferens
H01213  
Gallbladder disease
H01267  
Familial hyperinsulinemic hypoglycemia (HHF)
Drug
D00219  
Acetohexamide (JP17/USP/INN)
D00271  
Chlorpropamide (JP17/USP/INN)
D00294  
Diazoxide (JAN/USP/INN)
D00335  
Glipizide (USP/INN)
D00336  
Glyburide (USP)
D00379  
Tolazamide (JP17/USP/INN)
D00380  
Tolbutamide (JP17/USP/INN)
D00418  
Minoxidil (JAN/USP/INN)
D00593  
Glimepiride (JP17/USP/INN)
D00594  
Repaglinide (JAN/USP/INN)
D01111  
Nateglinide (JP17/USAN/INN)
D01356  
Glymidine sodium (JAN/USAN/INN)
D01599  
Gliclazide (JP17/INN)
D01799  
Glyclopyramide (JAN/INN)
D01810  
Nicorandil (JP17/USAN/INN)
D01854  
Mitiglinide calcium hydrate (JP17)
D02385  
Levcromakalim (USAN/INN)
D02425  
Carbutamide (INN)
D02427  
Glibornuride (USAN/INN)
D02430  
Gliquidone (INN)
D05482  
Pinacidil (USAN)
D06008  
Tariquidar (USAN/INN)
D06177  
Tolbutamide sodium, sterile
D06387  
Zosuquidar trihydrochloride (USAN)
D07117  
Metahexamide (INN)
D07118  
Glisoxepide (INN)
D09916  
Ivacaftor (USAN/INN)
D10134  
Lumacaftor (USAN)
Other DBs
Organism
Homo sapiens (human) [GN:hsa]
Gene
19  
ABCA1; ATP binding cassette subfamily A member 1 [KO:K05641]
20  
ABCA2; ATP binding cassette subfamily A member 2 [KO:K05642]
21  
ABCA3; ATP binding cassette subfamily A member 3 [KO:K05643]
24  
ABCA4; ATP binding cassette subfamily A member 4 [KO:K05644]
10347  
ABCA7; ATP binding cassette subfamily A member 7 [KO:K05645]
26154  
ABCA12; ATP binding cassette subfamily A member 12 [KO:K05646]
154664  
ABCA13; ATP binding cassette subfamily A member 13 [KO:K05647]
23461  
ABCA5; ATP binding cassette subfamily A member 5 [KO:K05648]
23460  
ABCA6; ATP binding cassette subfamily A member 6 [KO:K05649]
10351  
ABCA8; ATP binding cassette subfamily A member 8 [KO:K05650]
10350  
ABCA9; ATP binding cassette subfamily A member 9 [KO:K05651]
10349  
ABCA10; ATP binding cassette subfamily A member 10 [KO:K05652]
6890  
TAP1; transporter 1, ATP binding cassette subfamily B member [KO:K05653]
6891  
TAP2; transporter 2, ATP binding cassette subfamily B member [KO:K05654]
11194  
ABCB8; ATP binding cassette subfamily B member 8 [KO:K05655]
23457  
ABCB9; ATP binding cassette subfamily B member 9 [KO:K05656]
23456  
ABCB10; ATP binding cassette subfamily B member 10 [KO:K05657]
5243  
ABCB1; ATP binding cassette subfamily B member 1 [KO:K05658] [EC:3.6.3.44]
5244  
ABCB4; ATP binding cassette subfamily B member 4 [KO:K05659] [EC:3.6.3.44]
340273  
ABCB5; ATP binding cassette subfamily B member 5 [KO:K05660]
10058  
ABCB6; ATP binding cassette subfamily B member 6 (Langereis blood group) [KO:K05661]
22  
ABCB7; ATP binding cassette subfamily B member 7 [KO:K05662]
8647  
ABCB11; ATP binding cassette subfamily B member 11 [KO:K05664]
4363  
ABCC1; ATP binding cassette subfamily C member 1 [KO:K05665]
1244  
ABCC2; ATP binding cassette subfamily C member 2 [KO:K05666]
8714  
ABCC3; ATP binding cassette subfamily C member 3 [KO:K05667]
10257  
ABCC4; ATP binding cassette subfamily C member 4 [KO:K05673]
10057  
ABCC5; ATP binding cassette subfamily C member 5 [KO:K05668]
368  
ABCC6; ATP binding cassette subfamily C member 6 [KO:K05669]
multidrug resistance-associated protein 6 [KO:K05669]
89845  
ABCC10; ATP binding cassette subfamily C member 10 [KO:K05674]
85320  
ABCC11; ATP binding cassette subfamily C member 11 [KO:K05671]
94160  
ABCC12; ATP binding cassette subfamily C member 12 [KO:K05672]
1080  
CFTR; cystic fibrosis transmembrane conductance regulator [KO:K05031] [EC:3.6.3.49]
6833  
ABCC8; ATP binding cassette subfamily C member 8 [KO:K05032]
10060  
ABCC9; ATP binding cassette subfamily C member 9 [KO:K05033]
215  
ABCD1; ATP binding cassette subfamily D member 1 [KO:K05675]
225  
ABCD2; ATP binding cassette subfamily D member 2 [KO:K05676]
5825  
ABCD3; ATP binding cassette subfamily D member 3 [KO:K05677]
5826  
ABCD4; ATP binding cassette subfamily D member 4 [KO:K05678]
9619  
ABCG1; ATP binding cassette subfamily G member 1 [KO:K05679]
64137  
ABCG4; ATP binding cassette subfamily G member 4 [KO:K05680]
9429  
ABCG2; ATP binding cassette subfamily G member 2 (Junior blood group) [KO:K05681]
64240  
ABCG5; ATP binding cassette subfamily G member 5 [KO:K05683]
64241  
ABCG8; ATP binding cassette subfamily G member 8 [KO:K05684]
Compound
C00009  
Orthophosphate
C00025  
L-Glutamate
C00031  
D-Glucose
C00032  
Heme
C00034  
Manganese
C00037  
Glycine
C00038  
Zinc cation
C00041  
L-Alanine
C00045  
Amino acid
C00047  
L-Lysine
C00049  
L-Aspartate
C00051  
Glutathione
C00059  
Sulfate
C00062  
L-Arginine
C00064  
L-Glutamine
C00065  
L-Serine
C00070  
Copper
C00077  
L-Ornithine
C00079  
L-Phenylalanine
C00086  
Urea
C00088  
Nitrite
C00089  
Sucrose
C00093  
sn-Glycerol 3-phosphate
C00095  
D-Fructose
C00098  
Oligopeptide
C00107  
Dipeptide
C00114  
Choline
C00116  
Glycerol
C00120  
Biotin
C00121  
D-Ribose
C00123  
L-Leucine
C00134  
Putrescine
C00135  
L-Histidine
C00137  
myo-Inositol
C00140  
N-Acetyl-D-glucosamine
C00148  
L-Proline
C00151  
L-Amino acid
C00159  
D-Mannose
C00175  
Cobalt ion
C00181  
D-Xylose
C00183  
L-Valine
C00185  
Cellobiose
C00188  
L-Threonine
C00208  
Maltose
C00243  
Lactose
C00244  
Nitrate
C00245  
Taurine
C00259  
L-Arabinose
C00288  
HCO3-
C00291  
Nickel
C00315  
Spermidine
C00320  
Thiosulfate
C00333  
D-Galacturonate
C00338  
Lipopolysaccharide
C00378  
Thiamine
C00379  
Xylitol
C00392  
Mannitol
C00407  
L-Isoleucine
C00430  
5-Aminolevulinate
C00470  
Pectate
C00487  
Carnitine
C00491  
L-Cystine
C00492  
Raffinose
C00503  
Erythritol
C00719  
Betaine
C00753  
Tungsten
C00794  
D-Sorbitol
C00855  
D-Methionine
C00865  
Phospholipid
C00919  
Choline sulfate
C00973  
Cyclomaltodextrin
C01083  
alpha,alpha-Trehalose
C01153  
Orthophosphoric monoester
C01177  
Inositol 1-phosphate
C01181  
4-Trimethylammoniobutanoate
C01279  
4-Amino-5-hydroxymethyl-2-methylpyrimidine
C01330  
Sodium cation
C01417  
Cyanate
C01487  
D-Allose
C01606  
Phthalate
C01630  
Xylobiose
C01667  
Bacitracin
C01674  
Chitobiose
C01682  
Nopaline
C01684  
D-Rhamnose
C01834  
Lipoprotein
C01835  
Maltotriose
C01935  
Maltodextrin
C01946  
Oleandomycin
C02160  
Isomaltotriose
C02273  
Digalacturonate
C03557  
2-Aminoethylphosphonate
C03611  
Galactose oligosaccharide
C03619  
Methyl beta-D-galactoside
C04114  
(E)-4-(Trimethylammonio)but-2-enoate
C04137  
D-Octopine
C05349  
Ciprofloxacin
C05402  
Melibiose
C05776  
Vitamin B12
C06227  
Fe(III)hydroxamate
C06228  
Ferrichrome
C06229  
Fe(III)dicitrate
C06230  
Fe-enterobactin
C06232  
Molybdate
C06687  
Norfloxacin
C06704  
Iron chelate
C06705  
Capsular polysaccharide
C06706  
Lipo-oligosaccharide
C06707  
Teichoic acid
C06767  
Hemine
C07662  
Sparfloxacin
C07663  
Moxifloxacin
C11612  
Polymyxin B
C13768  
Colistin sulfate
C14818  
Fe2+
C14819  
Fe3+
C15521  
Alkanesulfonate
C16059  
Defensin beta-1
C16421  
AI-2
C16692  
Mannopine
C19609  
Nickel(2+)
C19872  
N-Formyl-4-amino-5-aminomethyl-2-methylpyrimidine
C20570  
alpha-1,5-L-Arabinobiose
C20571  
alpha-1,5-L-Arabinotriose
C20572  
alpha-1,5-L-Arabinotetraose
C20573  
Aldotetraouronic acid
D02224  
Protamine sulfate (JP17/USP/INN)
G00457  
Maltotetraose
Reference
PMID:9799792
  Authors
Tomii K, Kanehisa M.
  Title
A comparative analysis of ABC transporters in complete microbial genomes.
  Journal
Genome Res 8:1048-59 (1998)
Reference
  Authors
Sperandeo P, Lau FK, Carpentieri A, De Castro C, Molinaro A, Deho G, Silhavy TJ, Polissi A
  Title
Functional analysis of the protein machinery required for transport of lipopolysaccharide to the outer membrane of Escherichia coli.
  Journal
J Bacteriol 190:4460-9 (2008)
Reference
  Authors
Ruiz N, Gronenberg LS, Kahne D, Silhavy TJ
  Title
Identification of two inner-membrane proteins required for the transport of lipopolysaccharide to the outer membrane of Escherichia coli.
  Journal
Proc Natl Acad Sci U S A 105:5537-42 (2008)
Reference
  Authors
Omata T, Price GD, Badger MR, Okamura M, Gohta S, Ogawa T
  Title
Identification of an ATP-binding cassette transporter involved in bicarbonate uptake in the cyanobacterium Synechococcus sp. strain PCC 7942.
  Journal
Proc Natl Acad Sci U S A 96:13571-6 (1999)
Reference
  Authors
Quintero MJ, Montesinos ML, Herrero A, Flores E
  Title
Identification of genes encoding amino acid permeases by inactivation of selected ORFs from the Synechocystis genomic sequence.
  Journal
Genome Res 11:2034-40 (2001)
Reference
  Authors
Picossi S, Montesinos ML, Pernil R, Lichtle C, Herrero A, Flores E
  Title
ABC-type neutral amino acid permease N-I is required for optimal diazotrophic growth and is repressed in the heterocysts of Anabaena sp. strain PCC 7120.
  Journal
Mol Microbiol 57:1582-92 (2005)
Reference
  Authors
Valladares A, Montesinos ML, Herrero A, Flores E.
  Title
An ABC-type, high-affinity urea permease identified in cyanobacteria.
  Journal
Mol Microbiol 43:703-15 (2002)
Reference
  Authors
Richard-Fogal CL, Frawley ER, Kranz RG
  Title
Topology and function of CcmD in cytochrome c maturation.
  Journal
J Bacteriol 190:3489-93 (2008)
Reference
  Authors
Suzuki H, Koyanagi T, Izuka S, Onishi A, Kumagai H
  Title
The yliA, -B, -C, and -D genes of Escherichia coli K-12 encode a novel glutathione importer with an ATP-binding cassette.
  Journal
J Bacteriol 187:5861-7 (2005)
Reference
  Authors
Novikova M, Metlitskaya A, Datsenko K, Kazakov T, Kazakov A, Wanner B, Severinov K
  Title
The Escherichia coli Yej transporter is required for the uptake of translation inhibitor microcin C.
  Journal
J Bacteriol 189:8361-5 (2007)
Reference
  Authors
Tikhonova EB, Devroy VK, Lau SY, Zgurskaya HI
  Title
Reconstitution of the Escherichia coli macrolide transporter: the periplasmic membrane fusion protein MacA stimulates the ATPase activity of MacB.
  Journal
Mol Microbiol 63:895-910 (2007)
Reference
  Authors
Yamanaka H, Kobayashi H, Takahashi E, Okamoto K
  Title
MacAB is involved in the secretion of Escherichia coli heat-stable enterotoxin II.
  Journal
J Bacteriol 190:7693-8 (2008)
KO pathway
 

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