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P14314 Proteins LDL-receptor class A domain Repeats

Uniprot ID:P14314	Protein name: Glucosidase 2 subunit beta	Gene : PRKCSH G19P1
Protein Family:	Squence Length : 528

Sequnce	>P14314 529 MLLPLLLLLPMCWAVEVKRPRGVSLTNHHFYDESKPFTCLDGSATIPFDQVNDDYCDCKDGSDEPGTAACPNGSFHCTNTGYKPLYIPSNRVNDGVCDCCDGTDEYNSGVICENTCKEKGRKERESLQQMAEVTREGFRLKKILIEDWKKAREEKQKKLIELQAGKKSLEDQVEMLRTVKEEAEKPEREAKEQHQKLWEEQLAAAKAQQEQELAADAFKELDDDMDGTVSVTELQTHPELDTDGDGALSEAEAQALLSGDTQTDATSFYDRVWAAIRDKYRSEALPTDLPAPSAPDLTEPKEEQPPVPSSPTEEEEEEEEEEEEEAEEEEEEEDSEEAPPPLSPPQPASPAEEDKMPPYDEQTQAFIDAAQEARNKFEEAERSLKDMEESIRNLEQEISFDFGPNGEFAYLYSQCYELTTNEYVYRLCPFKLVSQKPKLGGSPTSLGTWGSWIGPDHDKFSAMKYEQGTGCWQGPNRSTTVRLLCGKETMVTSTTEPSRCEYLMELMTPAACPEPPPEAPTEDDHDEL
Domains	DOMAIN 37 71 LDL-receptor class A 1 DOMAIN 72 113 LDL-receptor class A 2 DOMAIN 209 244 EF-hand 1 DOMAIN 245 290 EF-hand 2 DOMAIN 413 468 PRKCSH
LDL-receptor class A sequence regions	37 - 71 CLDGSATIPFDQVNDDYCDCKDGSDEPGTAACPNG 72 - 113 SFHCTNTGYKPLYIPSNRVNDGVCDCCDGTDEYNSGVICENT
Function	"Regulatory subunit of glucosidase II that cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc(2)Man(9)GlcNAc(2) oligosaccharide precursor of immature glycoproteins"
Motifs	MOTIF 525 528 Prevents secretion from ER
Binding Site	BINDING 49 49 Substrate. shared with catalytic subunit. BINDING 53 53 Substrate. shared with catalytic subunit
Calcium Binding	CA_BIND 213 236 1
Disease	"DISEASE: Polycystic liver disease 1 with or without kidney cysts (PCLD1) [MIM:174050]: An autosomal dominant hepatobiliary disease characterized by overgrowth of biliary epithelium and supportive connective tissue, resulting in multiple liver cysts A subset of patients may develop kidney cysts that usually do not result in clinically significant renal disease Note=The disease is caused by mutations affecting the gene represented in this entry"