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
hsa02010  ABC transporters
hsa02010

Ortholog table
Disease
H00152  Sitosterolemia
H00159  Tangier disease
H00176  Adrenoleukodystrophy
H00208  Hyperbilirubinemia
H00218  Cystic fibrosis
H00560  Pseudoxanthoma elasticum
H00733  Harlequin ichthyosis
H00819  Stargardt disease
H00930  Hypoalphalipoproteinemia
H00984  Bare lymphocyte syndrome type1
H01033  Congenital bilateral absence of vas deferens
H01213  Gallbladder disease
H01267  Familial hyperinsulinemic hypoglycemia
H02056  Dubin-Johnson syndrome
Drug
D00219  Acetohexamide (JP17/USP/INN)
D00271  Chlorpropamide (JP17/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)
D03128  Biricodar dicitrate (USAN)
D03968  Elacridar hydrochloride (USAN)
D04739  Linogliride (USAN/INN)
D04740  Linogliride fumarate (USAN)
D05114  Naminidil (USAN/INN)
D05482  Pinacidil (USAN)
D06008  Tariquidar (USAN/INN)
D06084  Tesmilifene hydrochloride (USAN)
D06177  Tolbutamide sodium, sterile
D06277  Valspodar (USAN/INN)
D06387  Zosuquidar trihydrochloride (USAN)
D07117  Metahexamide (INN)
D07118  Glisoxepide (INN)
D10134  Lumacaftor (USAN)
D11041  Tezacaftor (USAN/INN)
D11402  Olacaftor (USAN)
Other DBs
BSID: 83035
GO: 0043190 0043191 0043192
TC: 3.A.1
Organism
Homo sapiens (human) [GN:hsa]
Gene
1672  DEFB1; defensin beta 1 [KO:K23125]
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:7.6.2.2]
5244  ABCB4; ATP binding cassette subfamily B member 4 [KO:K05659] [EC:7.6.2.2]
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] [EC:7.6.2.3]
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]
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
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
C01157  Hydroxyproline
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
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
C22040  S-Methyl-L-cysteine
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)
DOI:10.1101/gr.8.10.1048
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)
DOI:10.1128/JB.00270-08
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)
DOI:10.1073/pnas.0801196105
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)
DOI:10.1073/pnas.96.23.13571
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)
DOI:10.1101/gr.196301
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)
DOI:10.1111/j.1365-2958.2005.04791.x
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)
DOI:10.1046/j.1365-2958.2002.02778.x
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)
DOI:10.1128/JB.00146-08
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)
DOI:10.1128/JB.187.17.5861-5867.2005
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)
DOI:10.1128/JB.01028-07
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)
DOI:10.1111/j.1365-2958.2006.05549.x
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)
DOI:10.1128/JB.00853-08
KO pathway
ko02010   

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