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6LT0

cryo-EM structure of C9ORF72-SMCR8-WDR41

Functional Information from GO Data
ChainGOidnamespacecontents
A0005085molecular_functionguanyl-nucleotide exchange factor activity
A0005096molecular_functionGTPase activator activity
A0005515molecular_functionprotein binding
A0005737cellular_componentcytoplasm
A0005765cellular_componentlysosomal membrane
A0006914biological_processautophagy
A0010506biological_processregulation of autophagy
A0032045cellular_componentguanyl-nucleotide exchange factor complex
A0045920biological_processnegative regulation of exocytosis
A0050777biological_processnegative regulation of immune response
B0000785cellular_componentchromatin
B0004860molecular_functionprotein kinase inhibitor activity
B0005085molecular_functionguanyl-nucleotide exchange factor activity
B0005096molecular_functionGTPase activator activity
B0005515molecular_functionprotein binding
B0005634cellular_componentnucleus
B0005654cellular_componentnucleoplasm
B0005737cellular_componentcytoplasm
B0006351biological_processDNA-templated transcription
B0006914biological_processautophagy
B0010506biological_processregulation of autophagy
B0010629biological_processnegative regulation of gene expression
B0016242biological_processnegative regulation of macroautophagy
B0019901molecular_functionprotein kinase binding
B0032008biological_processpositive regulation of TOR signaling
B0032045cellular_componentguanyl-nucleotide exchange factor complex
B0045920biological_processnegative regulation of exocytosis
B0050777biological_processnegative regulation of immune response
B0098793cellular_componentpresynapse
B0098794cellular_componentpostsynapse
B1901098biological_processpositive regulation of autophagosome maturation
B1902902biological_processnegative regulation of autophagosome assembly
B1903432biological_processregulation of TORC1 signaling
B1990316cellular_componentAtg1/ULK1 kinase complex
C0000932cellular_componentP-body
C0001933biological_processnegative regulation of protein phosphorylation
C0005085molecular_functionguanyl-nucleotide exchange factor activity
C0005096molecular_functionGTPase activator activity
C0005515molecular_functionprotein binding
C0005576cellular_componentextracellular region
C0005615cellular_componentextracellular space
C0005634cellular_componentnucleus
C0005737cellular_componentcytoplasm
C0005764cellular_componentlysosome
C0005768cellular_componentendosome
C0005776cellular_componentautophagosome
C0005829cellular_componentcytosol
C0006897biological_processendocytosis
C0006914biological_processautophagy
C0010494cellular_componentcytoplasmic stress granule
C0010506biological_processregulation of autophagy
C0016239biological_processpositive regulation of macroautophagy
C0030424cellular_componentaxon
C0030425cellular_componentdendrite
C0030426cellular_componentgrowth cone
C0031267molecular_functionsmall GTPase binding
C0031410cellular_componentcytoplasmic vesicle
C0031965cellular_componentnuclear membrane
C0032045cellular_componentguanyl-nucleotide exchange factor complex
C0032880biological_processregulation of protein localization
C0034063biological_processstress granule assembly
C0043005cellular_componentneuron projection
C0043204cellular_componentperikaryon
C0044295cellular_componentaxonal growth cone
C0044304cellular_componentmain axon
C0044754cellular_componentautolysosome
C0048675biological_processaxon extension
C0061909biological_processautophagosome-lysosome fusion
C0098686cellular_componenthippocampal mossy fiber to CA3 synapse
C0098693biological_processregulation of synaptic vesicle cycle
C0098793cellular_componentpresynapse
C0098794cellular_componentpostsynapse
C0098978cellular_componentglutamatergic synapse
C0099523cellular_componentpresynaptic cytosol
C0110053biological_processregulation of actin filament organization
C1902774biological_processlate endosome to lysosome transport
C1903432biological_processregulation of TORC1 signaling
C1990316cellular_componentAtg1/ULK1 kinase complex
C2000785biological_processregulation of autophagosome assembly
D0005085molecular_functionguanyl-nucleotide exchange factor activity
D0005096molecular_functionGTPase activator activity
D0005515molecular_functionprotein binding
D0005737cellular_componentcytoplasm
D0005765cellular_componentlysosomal membrane
D0006914biological_processautophagy
D0010506biological_processregulation of autophagy
D0032045cellular_componentguanyl-nucleotide exchange factor complex
D0045920biological_processnegative regulation of exocytosis
D0050777biological_processnegative regulation of immune response
E0000785cellular_componentchromatin
E0004860molecular_functionprotein kinase inhibitor activity
E0005085molecular_functionguanyl-nucleotide exchange factor activity
E0005096molecular_functionGTPase activator activity
E0005515molecular_functionprotein binding
E0005634cellular_componentnucleus
E0005654cellular_componentnucleoplasm
E0005737cellular_componentcytoplasm
E0006351biological_processDNA-templated transcription
E0006914biological_processautophagy
E0010506biological_processregulation of autophagy
E0010629biological_processnegative regulation of gene expression
E0016242biological_processnegative regulation of macroautophagy
E0019901molecular_functionprotein kinase binding
E0032008biological_processpositive regulation of TOR signaling
E0032045cellular_componentguanyl-nucleotide exchange factor complex
E0045920biological_processnegative regulation of exocytosis
E0050777biological_processnegative regulation of immune response
E0098793cellular_componentpresynapse
E0098794cellular_componentpostsynapse
E1901098biological_processpositive regulation of autophagosome maturation
E1902902biological_processnegative regulation of autophagosome assembly
E1903432biological_processregulation of TORC1 signaling
E1990316cellular_componentAtg1/ULK1 kinase complex
F0000932cellular_componentP-body
F0001933biological_processnegative regulation of protein phosphorylation
F0005085molecular_functionguanyl-nucleotide exchange factor activity
F0005096molecular_functionGTPase activator activity
F0005515molecular_functionprotein binding
F0005576cellular_componentextracellular region
F0005615cellular_componentextracellular space
F0005634cellular_componentnucleus
F0005737cellular_componentcytoplasm
F0005764cellular_componentlysosome
F0005768cellular_componentendosome
F0005776cellular_componentautophagosome
F0005829cellular_componentcytosol
F0006897biological_processendocytosis
F0006914biological_processautophagy
F0010494cellular_componentcytoplasmic stress granule
F0010506biological_processregulation of autophagy
F0016239biological_processpositive regulation of macroautophagy
F0030424cellular_componentaxon
F0030425cellular_componentdendrite
F0030426cellular_componentgrowth cone
F0031267molecular_functionsmall GTPase binding
F0031410cellular_componentcytoplasmic vesicle
F0031965cellular_componentnuclear membrane
F0032045cellular_componentguanyl-nucleotide exchange factor complex
F0032880biological_processregulation of protein localization
F0034063biological_processstress granule assembly
F0043005cellular_componentneuron projection
F0043204cellular_componentperikaryon
F0044295cellular_componentaxonal growth cone
F0044304cellular_componentmain axon
F0044754cellular_componentautolysosome
F0048675biological_processaxon extension
F0061909biological_processautophagosome-lysosome fusion
F0098686cellular_componenthippocampal mossy fiber to CA3 synapse
F0098693biological_processregulation of synaptic vesicle cycle
F0098793cellular_componentpresynapse
F0098794cellular_componentpostsynapse
F0098978cellular_componentglutamatergic synapse
F0099523cellular_componentpresynaptic cytosol
F0110053biological_processregulation of actin filament organization
F1902774biological_processlate endosome to lysosome transport
F1903432biological_processregulation of TORC1 signaling
F1990316cellular_componentAtg1/ULK1 kinase complex
F2000785biological_processregulation of autophagosome assembly
Functional Information from PROSITE/UniProt
site_idPS00678
Number of Residues15
DetailsWD_REPEATS_1 Trp-Asp (WD) repeats signature. ILTAsaDrTVIVWDG
ChainResidueDetails
AILE106-GLY120
ALEU337-LEU351

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues78
DetailsRepeat: {"description":"WD 1"}
ChainResidueDetails

site_idSWS_FT_FI2
Number of Residues74
DetailsRepeat: {"description":"WD 3"}
ChainResidueDetails

site_idSWS_FT_FI3
Number of Residues76
DetailsRepeat: {"description":"WD 6"}
ChainResidueDetails

site_idSWS_FT_FI4
Number of Residues132
DetailsDomain: {"description":"dDENN FLCN/SMCR8-type","evidences":[{"source":"PROSITE-ProRule","id":"PRU01178","evidenceCode":"ECO:0000255"},{"source":"PubMed","id":"23248642","evidenceCode":"ECO:0000305"}]}
ChainResidueDetails

site_idSWS_FT_FI5
Number of Residues104
DetailsRegion: {"description":"Interacts with WDR41 within the C9orf72-SMCR8 complex","evidences":[{"source":"PubMed","id":"32303654","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI6
Number of Residues2
DetailsModified residue: {"description":"Phosphothreonine; by TBK1","evidences":[{"source":"PubMed","id":"27103069","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI7
Number of Residues188
DetailsDomain: {"description":"dDENN C9ORF72-type","evidences":[{"source":"PROSITE-ProRule","id":"PRU01179","evidenceCode":"ECO:0000255"}]}
ChainResidueDetails

247536

PDB entries from 2026-01-14

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