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Cystinuria Autosomal recessive mutation in membrane transport protein. Defect in transport of cystine from fluid of proximal convoluted tubules of the kidneys. Get kidney stones rich in cystine. Defective genes: SLC3A1 and SLC7A9 which code for high affinity cystine transporter present in the apical brush border membrane of the jejunum Valinomycin cyclin peptide ionophore that transports K+ down its electrochemical gradient FCCP ionophore which makes membrane leaky to H+ A23187 ionophore which makes membrane leaky to divalent cations like Mg2+ and Ca2+, used widely to increase intracellular concentration of Ca2+, mimicking signalling pathways that signal Ca2+ release Gramicidin A ionophore of 2 15AA subunits that forms a channel that lets monovalent cations like H+, K+ and Na+ pass through down their electrochemical gradients GLUT1-4 uniporter, passive glucose transporter, involved in glucose re-absorption in the kidney /gut SGLT1,4 symporter/cotransporter, sodium glucose transporters, utilizes sodium gradient for glucose re-absorption in the kidney/but. NHE1-4 antiporter/exchanger, sodium-proton exchanger, utilizes sodium gradient for deacidification KCC2 Cl cotransporter which uses potassium gradient already established by Na/K ATPase to drive chlorines out, allowing Cl ions to move into cell through channel proteins. In adult, low Cl in cell, in development high Cl in cell because it is not expressed in development, so high intracellular Cl concentrations P type ATPase phosphorylation type, ATP driven cation transporters, reversibly phosphorylated by ATP as part of the transport mechanism. They become phosphorylated and then de-phosphorylated. V type ATPase vascuolar type, exclusively proton pumps, acidify contents of intracellular compartments, large multisubunit ATPases F type ATPase identical to V-type but in opposite direction, energy transducing reactions in mitochondria in mammalian cells, , convert potential energy of H+ electrochemical gradient into chemical bond energy, they run ATP hydrolysis backwards, so they're responsible for ATP synthesis ABC pumps ATPase which transport small molecules across membranes. Transport phospholipids and lipophilic drugs also in mammalian cells aspartyl phosphate intermediate P type ATPases form this intermediate