Function
Component of the C9orf72-SMCR8 complex, a complex that has guanine nucleotide exchange factor (GEF) activity and regulates autophagy. In the complex, C9orf72 and SMCR8 probably constitute the catalytic subunits that promote the exchange of GDP to GTP, converting inactive GDP-bound RAB8A and RAB39B into their active GTP-bound form, thereby promoting autophagosome maturation. The C9orf72-SMCR8 complex also acts as a negative regulator of autophagy initiation by interacting with the ATG1/ULK1 kinase complex and inhibiting its protein kinase activity.
Sequence
MIGSPDLVAFTKETDFSEITTDSSVLPEDLSVPMYPYTGDATPWSKISSAKLKKDFILISEFSEQVGPQPLLTVPLETKACGTFDLNYFSLRIMSVDYQTSLAGSPGYGSFKLNFVEDSKVVLADSREGVFAYVHHLTLYDLEARGFVRPLCLAYVSSDENKIIQQFQRISTEFNKVSECLKTGNRKNFANELEVKLRDLEYTRVVLQKELNTVSVKCSSEREPILNGVHSFERNADEVKLNEKSSHTDEISPQEKDGCGNSRKVEVKLENENRSHFEHEQYGKQRKDKPDKTSCPMPLANKNDELASVEKLIQDYKSLLKQVTCYPTRKLRDSEYSPYEPDDLPQSFDLDLDSQFAGPMLECSVFTYTNTPSQTLQQINSTSSSRFDKRLKTLEELCDDYFYQQALQQLYSIERTFRGDACYLYTQQLCRNLLRNLKSTNFLFEDPCDLDDDVGLQIGQSTIQQPSFLPAPSFLSGPVSLESYASCVEMVPIKLELGGSSQSQVQHSTLNTPSKDNRPQVADKSPAEVEMKGEIISAPDCQGNVESVSNLMKTSISSGDSIEVLGTERSFRSQGANTLVETAMHRPPPLSSATALEGLKQGRVPTRRTCSEDSIEVLCITESISPDELRASYPCAIDEESPEQETDEKNSSQYQEDNNEKSIYVQGKISADHENACLKKLHPSVTVTPPDCPLTLEETSFQDSCQATESATMLLLDEPSRMVPDDLSDCFSYRSTTASTTSECTFPACLPKDKREGGTRRRRGRVGRAALQFMRQFPFAVHAVFSLLSGRTLVVLGSEEAAVRRLVTALSVYLPHLTKYKDSIQPWTSTPLQLTDLLNWKLIGFDRMCSFNPSSLPHCLDHYSRYLSILDVDQKTLHCPTYSGSLINLLVEPKSHFKRGNTYFTFAQSVQSKLVTKAFLLTFSHGHPSPSRPQGSSGTECFLSELHTDDKKILRYLSELIKLHFMEVTPNVLLFSYTTTSIFKL