ABC transporter disorders

Tarling EJ, Vallim TQDA, Edwards PA. Role of ABC transporters in lipid transport and human disease. Trends in Endocrinology & Metabolism. 2013 Jul;24(7):342–50. doi: 10.1016/j.tem.2013.01.006.; Kemp S, Theodoulou FL, Wanders RJ. Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance. British J Pharmacology. 2011 Nov 15;164(7):1753–66. doi: 10.1111/j.1476-5381.2011.01435.x.; Chen Z, Tiwari AK. Multidrug resistance proteins (MRPs/ABCCs) in cancer chemotherapy and genetic diseases. The FEBS Journal. 2011 Aug;278(18):3226–45. doi: 10.1111/j.1742-4658.2011.08235.x.; Woodward OM, Köttgen A, Köttgen M. ABCG transporters and disease. The FEBS Journal. 2011 Jun 13;278(18):3215–25. doi: 10.1111/j.1742-4658.2011.08171.x.; UEDA K. ABC Proteins Protect the Human Body and Maintain Optimal Health. Bioscience, Biotechnology, and Biochemistry. 2011 Mar 23;75(3):401–9. doi: 10.1271/bbb.100816.; Dean M. The genetics of ATP-binding cassette transporters. Methods Enzymol. 2005;400():409–29. doi: 10.1016/s0076-6879(05)00024-8. PMID: 16399363.

Metabolites

Cholesterol

Formula: C27H46O (386.3548466)

CAS ID: 57-88-5

Cl-

Formula: Cl (34.968853)

CAS ID: 16887-00-6

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Porphyrin

Formula: C20H14N4 (310.1218404)

CAS ID: 101-60-0

Potassium cation

Formula: K (38.963708)

CAS ID: 24203-36-9

Ivacaftor

Formula: C24H28N2O3 (392.20998180000004)

CAS ID: 873054-44-5

ATP(4-)

Formula: C10H12N5O13P3 (502.9644492)

CAS ID:

ADP(3-)

Formula: C10H12N5O10P2 (424.0059412)

CAS ID:



Enzyme

EC Number name full name note
7.6.2.-
3.6.4.6 vesicle-fusing ATPase ATP phosphohydrolase (vesicle-fusing)
5.6.1.6 channel-conductance-controlling ATPase ATP phosphohydrolase (channel-conductance-controlling)
7.6.2.3 ABC-type glutathione-S-conjugate transporter ATP + H2O + glutathione-S-conjugate[side 1] = ADP + phosphate + glutathione-S-conjugate[side 2]
7.6.2.8 ABC-type vitamin B12 transporter ATP phosphohydrolase (ABC-type, vitamin B12-importing)


Proteins

Protein ID name full name