13, 34, 53116917, 21, 49, 75, 8026, 586411, 4215, 672, 4, 15, 37, 42...435, 357, 7017, 21, 49, 75, 8024, 30, 38, 73, 816, 23, 50, 7829, 46, 65, 6632, 51, 773, 20, 33, 47, 55...6, 23, 50, 7827, 28, 4140, 5612, 741, 8, 1614, 22, 25, 31, 59...1519, 48769, 18, 546124, 36, 39, 45, 6344, 57transport vesiclelysosomal lumenperoxisomal matrixlamellar bodymitochondrial matrixcytosolendoplasmic reticulum lumenH2ODERL3 ATPADPABCG5 APOA1(25-266) OS9 ADPEIF2S3 UBC(381-456) atRALATPPiH2OABCA7-dependentphospholipidsADPADPADPmisfolded CFTR ABCD1 PSMD14 PC PC, PGABCD2 PiLCFAPG PSMC5 ABCD2 DERL3 ABCB9 dimerERLIN1 UBB(1-76) ERLEC1 UBA52(1-76) ATPATPUBB(77-152) EIF2S2 PSMA6 PSMD7 H2OABCA4H2OATPERLIN2 PSMC4 ATPPSMC3 misfolded CFTR:ERADcomplexABCA12PSMD5 lipidslipidsCHOLUBA52(1-76) PSMD6 ABCC9 UBC(609-684) ADPCl-PiPiABCF1:EIF2S1:EIF2S2:EIF2S3PiatRALUBC(609-684) H2ORNF5 UBC(77-152) PiRPS27A(1-76) H2OUBC(153-228) UBC(457-532) PSMD9 ADPATPPSME4 OS9 CFTR G551DPEX19 ABCB9 PSMB3 PiADPUBB(1-76) PiABCA7-1:ApoA1complexBMG,BDGATPERAD complexRNF5 ATPUBC(1-76) PSMD2 ABCG1 dimerhemeUBC(1-76) PiH2ORPS27A(1-76) Ub-misfolded nascentCFTRABCC11 a xenobioticPia xenobioticUBC(153-228) ABCA10 UBB(1-76) UBC(533-608) IvacaftorABCB1 PEX3ERLIN1 PSMD13 PSMC2 Cl-PSMD3 H2OCHOLCHOL PSMA2 ABCC9 CFTR G551D UBC(381-456) ATPABCA2 BMG OS9 UbH2OsterolsBMG,BDGABCC3 UBC(77-152) PSMD11 UBA52(1-76) ATPPiPSMB9 UBC(609-684) KCNJ11:ABCC9PSMC6 PSMD4 ATPABCB6 UBC(77-152) PiUBA52(1-76) ATPIvacaftor:CFTR G551DPiSPHM HCO3-UBC(305-380) RNF185 UBC(533-608) ABCC2 ABCCsPSMB7 sterolsDERL1 H2OEIF2S2 PSME1 H2OPSMA4 PSMB8 CHOLPSME3 SEL1L UBC(1-76) ABCA cholesteroltransportersUBB(153-228) PEX19:ABCD1/2/3ERLIN1 PCporphyrinUBC(229-304) PSMD10 ERLIN2 UBB(77-152) UBC(1-76) PSMA3 PiCl-ATPCHOLABCC6 UBC(305-380) CHOL ABCG1 misfolded CFTR PSMB6 ADPPSMF1 PSMA8 VCP SPHM ABCC10 CHOLABCG4 dimerABCA8/B1/B5PSMD1 UBB(77-152) BMG K+porphyrinphytosterols CFTRPiATPUBB(153-228) PSMD8 CHOLDERL2 K+PC PSMB10 PSMB5 UBC(381-456) PSMA7 ADPVCP RNF185 UBC(457-532) ATPUBB(153-228) UBB(1-76) PSMB1 PG ABCA9 ERLEC1 H2OCFTRADPUBC(457-532) PSME2 Ivacaftor H2OUBB(77-152) PiDERL2 ABCA7-dependentphospholipidsRNF185 EIF2S1 23mer peptideABCC4 RNF5 H2Ophytosterols ABCD3 misfolded CFTRPSMD12 misfolded CFTR PCVCP UbCHOLADPABCG5:ABCG8PSMB11 H2OPiPiDERL2 PiADPDERL1 ABCF1 UBC(457-532) Heme transportersUBC(381-456) ABCC1 PC UBC(77-152) ABCB5 ABCB10 UBC(229-304) UBC(305-380) DERL3 23mer peptidePC ABCD3 DERL1 PSMC1 26S proteasomeUb-misfolded nascentCFTR:ERAD complexUBB(153-228) H2ORPS27A(1-76) organic anionABCB8 ABCA5SEL1L EIF2S1:EIF2S2:EIF2S3UBC(229-304) UBC(305-380) RPS27A(1-76) ADPMTABC3 dimerABCA3SHFM1 hemeATPUBC(229-304) ATPERLEC1 ERLIN2 ADPUBC(533-608) PEX19PSMB2 ABCD1 ADPKCNJ11 LCFAPSMA5 organic anionABCD1-3 dimersEIF2S3 UBC(609-684) ABCG8 CFTR ADPADPUBC(153-228) ABCB4ABCC2ABCB7(23-752) HCO3-UBC(153-228) UBC(533-608) ADPABCG4 ABCA8 ABCF1SEL1L H2OPSMB4 EIF2S1 ABCC5 PSMA1 ABCA6 ABCA7 PC, PGBDG H2OBDG 10, 5210