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Protein

Glucosidase 2 subunit beta

Gene

PRKCSH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Regulatory subunit of glucosidase II.1 Publication

Pathwayi: N-glycan metabolism

This protein is involved in the pathway N-glycan metabolism, which is part of Glycan metabolism.
View all proteins of this organism that are known to be involved in the pathway N-glycan metabolism and in Glycan metabolism.

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Calcium bindingi213 – 2361PROSITE-ProRule annotationAdd BLAST24

GO - Molecular functioni

  • calcium ion binding Source: InterPro
  • ion channel binding Source: UniProtKB
  • phosphoprotein binding Source: UniProtKB
  • protein kinase C binding Source: UniProtKB

GO - Biological processi

  • intracellular signal transduction Source: UniProtKB
  • N-glycan processing Source: InterPro
  • protein folding Source: Reactome
Complete GO annotation...

Keywords - Ligandi

Calcium, Metal-binding

Enzyme and pathway databases

BioCyciZFISH:HS05346-MONOMER.
BRENDAi3.2.1.84. 2681.
ReactomeiR-HSA-532668. N-glycan trimming in the ER and Calnexin/Calreticulin cycle.
R-HSA-879415. Advanced glycosylation endproduct receptor signaling.
R-HSA-901042. Calnexin/calreticulin cycle.
SignaLinkiP14314.
UniPathwayiUPA00957.

Names & Taxonomyi

Protein namesi
Recommended name:
Glucosidase 2 subunit beta
Alternative name(s):
80K-H protein
Glucosidase II subunit beta
Protein kinase C substrate 60.1 kDa protein heavy chain
Short name:
PKCSH
Gene namesi
Name:PRKCSH
Synonyms:G19P1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:9411. PRKCSH.

Subcellular locationi

  • Endoplasmic reticulum PROSITE-ProRule annotation

GO - Cellular componenti

  • endoplasmic reticulum Source: UniProtKB
  • endoplasmic reticulum lumen Source: Reactome
  • intracellular Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum

Pathology & Biotechi

Involvement in diseasei

Polycystic liver disease 1 (PCLD1)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal dominant hepatobiliary disease characterized by overgrowth of biliary epithelium and supportive connective tissue, resulting in multiple liver cysts.
See also OMIM:174050

Organism-specific databases

DisGeNETi5589.
MalaCardsiPRKCSH.
MIMi174050. phenotype.
OpenTargetsiENSG00000130175.
Orphaneti2924. Isolated polycystic liver disease.
PharmGKBiPA33774.

Polymorphism and mutation databases

BioMutaiPRKCSH.
DMDMi116242499.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 141 PublicationAdd BLAST14
ChainiPRO_000000414315 – 528Glucosidase 2 subunit betaAdd BLAST514

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei24Phosphoserine; by FAM20C1 Publication1
Glycosylationi72N-linked (GlcNAc...)Sequence analysis1
Modified residuei89Phosphoserine; by PKCSequence analysis1
Modified residuei166N6-succinyllysineBy similarity1
Modified residuei168Phosphoserine; by FAM20C1 Publication1
Modified residuei383Phosphoserine; by PKCSequence analysis1
Modified residuei390Phosphoserine; by PKCSequence analysis1
Modified residuei434Phosphoserine; by PKCSequence analysis1
Glycosylationi476N-linked (GlcNAc...)Sequence analysis1

Keywords - PTMi

Glycoprotein, Phosphoprotein

Proteomic databases

EPDiP14314.
MaxQBiP14314.
PaxDbiP14314.
PeptideAtlasiP14314.
PRIDEiP14314.
TopDownProteomicsiP14314-1. [P14314-1]
P14314-2. [P14314-2]

PTM databases

iPTMnetiP14314.
PhosphoSitePlusiP14314.
SwissPalmiP14314.

Miscellaneous databases

PMAP-CutDBP14314.

Expressioni

Gene expression databases

BgeeiENSG00000130175.
CleanExiHS_PRKCSH.
ExpressionAtlasiP14314. baseline and differential.
GenevisibleiP14314. HS.

Organism-specific databases

HPAiCAB004465.
HPA041940.
HPA043560.

Interactioni

Subunit structurei

Heterodimer of a catalytic alpha subunit (GANAB) and a beta subunit (PRKCSH). Binds glycosylated PTPRC (By similarity).By similarity

GO - Molecular functioni

  • ion channel binding Source: UniProtKB
  • phosphoprotein binding Source: UniProtKB
  • protein kinase C binding Source: UniProtKB

Protein-protein interaction databases

BioGridi111575. 55 interactors.
IntActiP14314. 19 interactors.
MINTiMINT-1380114.
STRINGi9606.ENSP00000252455.

Structurei

3D structure databases

ProteinModelPortaliP14314.
SMRiP14314.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini209 – 244EF-hand 1PROSITE-ProRule annotationAdd BLAST36
Domaini245 – 290EF-hand 2PROSITE-ProRule annotationAdd BLAST46
Domaini413 – 468PRKCSHAdd BLAST56

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi525 – 528Prevents secretion from ERPROSITE-ProRule annotation4

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi313 – 336Glu-rich (acidic)Add BLAST24

Sequence similaritiesi

Contains 2 EF-hand domains.PROSITE-ProRule annotation
Contains 1 PRKCSH domain.Curated

Keywords - Domaini

Repeat, Signal

Phylogenomic databases

eggNOGiKOG2397. Eukaryota.
ENOG410XPE3. LUCA.
GeneTreeiENSGT00510000047770.
HOGENOMiHOG000007805.
HOVERGENiHBG051738.
InParanoidiP14314.
KOiK08288.
PhylomeDBiP14314.
TreeFamiTF329550.

Family and domain databases

Gene3Di2.70.130.10. 1 hit.
4.10.400.10. 2 hits.
InterProiIPR011992. EF-hand-dom_pair.
IPR018247. EF_Hand_1_Ca_BS.
IPR002048. EF_hand_dom.
IPR026874. Glucosidase_2_bsu.
IPR002172. LDrepeatLR_classA_rpt.
IPR009011. Man6P_isomerase_rcpt-bd_dom.
IPR028146. PRKCSH_N.
[Graphical view]
PANTHERiPTHR12630:SF1. PTHR12630:SF1. 1 hit.
PfamiPF13202. EF-hand_5. 1 hit.
PF12999. PRKCSH-like. 1 hit.
PF13015. PRKCSH_1. 1 hit.
[Graphical view]
SUPFAMiSSF47473. SSF47473. 1 hit.
SSF50911. SSF50911. 1 hit.
SSF57424. SSF57424. 1 hit.
PROSITEiPS00018. EF_HAND_1. 1 hit.
PS50222. EF_HAND_2. 1 hit.
PS00014. ER_TARGET. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P14314-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MLLPLLLLLP MCWAVEVKRP RGVSLTNHHF YDESKPFTCL DGSATIPFDQ
60 70 80 90 100
VNDDYCDCKD GSDEPGTAAC PNGSFHCTNT GYKPLYIPSN RVNDGVCDCC
110 120 130 140 150
DGTDEYNSGV ICENTCKEKG RKERESLQQM AEVTREGFRL KKILIEDWKK
160 170 180 190 200
AREEKQKKLI ELQAGKKSLE DQVEMLRTVK EEAEKPEREA KEQHQKLWEE
210 220 230 240 250
QLAAAKAQQE QELAADAFKE LDDDMDGTVS VTELQTHPEL DTDGDGALSE
260 270 280 290 300
AEAQALLSGD TQTDATSFYD RVWAAIRDKY RSEALPTDLP APSAPDLTEP
310 320 330 340 350
KEEQPPVPSS PTEEEEEEEE EEEEEAEEEE EEEDSEEAPP PLSPPQPASP
360 370 380 390 400
AEEDKMPPYD EQTQAFIDAA QEARNKFEEA ERSLKDMEES IRNLEQEISF
410 420 430 440 450
DFGPNGEFAY LYSQCYELTT NEYVYRLCPF KLVSQKPKLG GSPTSLGTWG
460 470 480 490 500
SWIGPDHDKF SAMKYEQGTG CWQGPNRSTT VRLLCGKETM VTSTTEPSRC
510 520
EYLMELMTPA ACPEPPPEAP TEDDHDEL
Length:528
Mass (Da):59,425
Last modified:October 17, 2006 - v2
Checksum:i8DAD9776037E878E
GO
Isoform 2 (identifier: P14314-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     337-346: EAPPPLSPPQ → VQGEQPK

Show »
Length:525
Mass (Da):59,178
Checksum:iABACF156C534F30E
GO

Sequence cautioni

The sequence AAH15154 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti325Missing in AAA52493 (PubMed:2793184).Curated1
Sequence conflicti325Missing in AAA98668 (PubMed:9043864).Curated1
Sequence conflicti325Missing in AAP88860 (Ref. 4) Curated1
Sequence conflicti325Missing in AAH13586 (PubMed:15057824).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02876174S → N.Corresponds to variant rs10406672dbSNPEnsembl.1
Natural variantiVAR_028762291A → T.2 PublicationsCorresponds to variant rs11557488dbSNPEnsembl.1
Natural variantiVAR_048658338A → G.Corresponds to variant rs35847588dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_043749337 – 346EAPPPLSPPQ → VQGEQPK in isoform 2. 2 Publications10

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J03075 mRNA. Translation: AAA52493.1.
U50326
, U50317, U50318, U50319, U50320, U50321, U50322, U50323, U50324, U50325 Genomic DNA. Translation: AAA98668.1.
AF144075 mRNA. Translation: AAF66686.1.
BT009858 mRNA. Translation: AAP88860.1.
AK290433 mRNA. Translation: BAF83122.1.
AC008481 Genomic DNA. No translation available.
AC024575 Genomic DNA. No translation available.
BC013586 mRNA. Translation: AAH13586.2.
BC015154 mRNA. Translation: AAH15154.1. Different initiation.
CCDSiCCDS32911.1. [P14314-1]
CCDS45977.1. [P14314-2]
PIRiA32469.
RefSeqiNP_001001329.1. NM_001001329.2. [P14314-2]
NP_001276031.1. NM_001289102.1. [P14314-2]
NP_002734.2. NM_002743.3. [P14314-1]
XP_011526433.1. XM_011528131.1. [P14314-1]
XP_011526434.1. XM_011528132.1. [P14314-2]
XP_016882466.1. XM_017026977.1. [P14314-1]
UniGeneiHs.610830.

Genome annotation databases

EnsembliENST00000587327; ENSP00000466012; ENSG00000130175. [P14314-2]
ENST00000589838; ENSP00000465461; ENSG00000130175. [P14314-1]
ENST00000591462; ENSP00000465489; ENSG00000130175. [P14314-2]
GeneIDi5589.
KEGGihsa:5589.
UCSCiuc010dyb.5. human. [P14314-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J03075 mRNA. Translation: AAA52493.1.
U50326
, U50317, U50318, U50319, U50320, U50321, U50322, U50323, U50324, U50325 Genomic DNA. Translation: AAA98668.1.
AF144075 mRNA. Translation: AAF66686.1.
BT009858 mRNA. Translation: AAP88860.1.
AK290433 mRNA. Translation: BAF83122.1.
AC008481 Genomic DNA. No translation available.
AC024575 Genomic DNA. No translation available.
BC013586 mRNA. Translation: AAH13586.2.
BC015154 mRNA. Translation: AAH15154.1. Different initiation.
CCDSiCCDS32911.1. [P14314-1]
CCDS45977.1. [P14314-2]
PIRiA32469.
RefSeqiNP_001001329.1. NM_001001329.2. [P14314-2]
NP_001276031.1. NM_001289102.1. [P14314-2]
NP_002734.2. NM_002743.3. [P14314-1]
XP_011526433.1. XM_011528131.1. [P14314-1]
XP_011526434.1. XM_011528132.1. [P14314-2]
XP_016882466.1. XM_017026977.1. [P14314-1]
UniGeneiHs.610830.

3D structure databases

ProteinModelPortaliP14314.
SMRiP14314.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111575. 55 interactors.
IntActiP14314. 19 interactors.
MINTiMINT-1380114.
STRINGi9606.ENSP00000252455.

PTM databases

iPTMnetiP14314.
PhosphoSitePlusiP14314.
SwissPalmiP14314.

Polymorphism and mutation databases

BioMutaiPRKCSH.
DMDMi116242499.

Proteomic databases

EPDiP14314.
MaxQBiP14314.
PaxDbiP14314.
PeptideAtlasiP14314.
PRIDEiP14314.
TopDownProteomicsiP14314-1. [P14314-1]
P14314-2. [P14314-2]

Protocols and materials databases

DNASUi5589.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000587327; ENSP00000466012; ENSG00000130175. [P14314-2]
ENST00000589838; ENSP00000465461; ENSG00000130175. [P14314-1]
ENST00000591462; ENSP00000465489; ENSG00000130175. [P14314-2]
GeneIDi5589.
KEGGihsa:5589.
UCSCiuc010dyb.5. human. [P14314-1]

Organism-specific databases

CTDi5589.
DisGeNETi5589.
GeneCardsiPRKCSH.
HGNCiHGNC:9411. PRKCSH.
HPAiCAB004465.
HPA041940.
HPA043560.
MalaCardsiPRKCSH.
MIMi174050. phenotype.
177060. gene.
neXtProtiNX_P14314.
OpenTargetsiENSG00000130175.
Orphaneti2924. Isolated polycystic liver disease.
PharmGKBiPA33774.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2397. Eukaryota.
ENOG410XPE3. LUCA.
GeneTreeiENSGT00510000047770.
HOGENOMiHOG000007805.
HOVERGENiHBG051738.
InParanoidiP14314.
KOiK08288.
PhylomeDBiP14314.
TreeFamiTF329550.

Enzyme and pathway databases

UniPathwayiUPA00957.
BioCyciZFISH:HS05346-MONOMER.
BRENDAi3.2.1.84. 2681.
ReactomeiR-HSA-532668. N-glycan trimming in the ER and Calnexin/Calreticulin cycle.
R-HSA-879415. Advanced glycosylation endproduct receptor signaling.
R-HSA-901042. Calnexin/calreticulin cycle.
SignaLinkiP14314.

Miscellaneous databases

ChiTaRSiPRKCSH. human.
GeneWikiiPRKCSH.
GenomeRNAii5589.
PMAP-CutDBP14314.
PROiP14314.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000130175.
CleanExiHS_PRKCSH.
ExpressionAtlasiP14314. baseline and differential.
GenevisibleiP14314. HS.

Family and domain databases

Gene3Di2.70.130.10. 1 hit.
4.10.400.10. 2 hits.
InterProiIPR011992. EF-hand-dom_pair.
IPR018247. EF_Hand_1_Ca_BS.
IPR002048. EF_hand_dom.
IPR026874. Glucosidase_2_bsu.
IPR002172. LDrepeatLR_classA_rpt.
IPR009011. Man6P_isomerase_rcpt-bd_dom.
IPR028146. PRKCSH_N.
[Graphical view]
PANTHERiPTHR12630:SF1. PTHR12630:SF1. 1 hit.
PfamiPF13202. EF-hand_5. 1 hit.
PF12999. PRKCSH-like. 1 hit.
PF13015. PRKCSH_1. 1 hit.
[Graphical view]
SUPFAMiSSF47473. SSF47473. 1 hit.
SSF50911. SSF50911. 1 hit.
SSF57424. SSF57424. 1 hit.
PROSITEiPS00018. EF_HAND_1. 1 hit.
PS50222. EF_HAND_2. 1 hit.
PS00014. ER_TARGET. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiGLU2B_HUMAN
AccessioniPrimary (citable) accession number: P14314
Secondary accession number(s): A8K318
, Q96BU9, Q96D06, Q9P0W9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 1, 1990
Last sequence update: October 17, 2006
Last modified: November 2, 2016
This is version 181 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.