Conserved Intramolecular Disulfide Bond Is Critical to Trafficking and Fate of ATP-binding Cassette (ABC) Transporters ABCB6 and Sulfonylurea Receptor 1 (SUR1)/ABCC8

The ATP-binding cassette (ABC) transporter ABCB6 is a mitochondrial porphyrin transporter that activates porphyrin biosynthesis. ABCB6 lacks a canonical mitochondrial targeting sequence but reportedly traffics to other cellular compartments such as the plasma membrane. How ABCB6 reaches these destin...

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Veröffentlicht in:	The Journal of biological chemistry 2011-03, Vol.286 (10), p.8481-8492
Hauptverfasser:	Fukuda, Yu, Aguilar-Bryan, Lydia, Vaxillaire, Martine, Dechaume, Aurelie, Wang, Yao, Dean, Michael, Moitra, Karobi, Bryan, Joseph, Schuetz, John D.
Format:	Artikel
Sprache:	eng
Schlagworte:	ABC Transporter Alleles Amino Acid Substitution Animals ATP-Binding Cassette Transporters - genetics ATP-Binding Cassette Transporters - metabolism Carbohydrate Metabolism, Inborn Errors - genetics Carbohydrate Metabolism, Inborn Errors - metabolism Cell Biology Cysteine Proteinase Inhibitors - pharmacology Disulfide Disulfides - metabolism Endoplasmic Reticulum - genetics Endoplasmic Reticulum - metabolism Glycosylation HEK293 Cells Humans Hyperinsulinism - genetics Hyperinsulinism - metabolism Hypoglycemia - genetics Hypoglycemia - metabolism K562 Cells Leupeptins - pharmacology Membrane Proteins Mice Mitochondrial Proteins - genetics Mitochondrial Proteins - metabolism Mutation, Missense NIH 3T3 Cells Potassium Channels, Inwardly Rectifying - genetics Potassium Channels, Inwardly Rectifying - metabolism Proteasome Endopeptidase Complex - genetics Proteasome Endopeptidase Complex - metabolism Proteasome Inhibitors Protein Degradation Protein Folding Protein Motifs Protein Stability Protein Structure, Tertiary Protein Transport - genetics Receptors, Drug - genetics Receptors, Drug - metabolism Sulfonylurea Receptors
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