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Protein

Endoplasmic reticulum protein SC65

Gene

P3h4

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linking of collagen fibrils (PubMed:27119146). Required for normal bone density and normal skin stability via its role in hydroxylation of lysine residues in collagen alpha chains and in collagen fibril assembly (PubMed:23959653, PubMed:27119146, PubMed:28115524).3 Publications

GO - Biological processi

  • bone remodeling Source: UniProtKB
  • collagen biosynthetic process Source: UniProtKB
  • collagen fibril organization Source: UniProtKB
  • peptidyl-lysine hydroxylation Source: UniProtKB

Names & Taxonomyi

Protein namesi
Recommended name:
Endoplasmic reticulum protein SC651 Publication
Alternative name(s):
Leprecan-like protein 4
Prolyl 3-hydroxylase family member 4Imported
Synaptonemal complex protein SC65By similarity
Gene namesi
Name:P3h4Imported
Synonyms:Leprel4, Sc65By similarity
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 11

Organism-specific databases

MGIiMGI:1913430 P3h4

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Endoplasmic reticulum

Pathology & Biotechi

Disruption phenotypei

No visible phenotype at birth (PubMed:23959653, PubMed:27119146, PubMed:28115524). Mice are born at the expected Mendelian rate (PubMed:23959653, PubMed:27119146). Two and six month old mutant mice display a decreased ratio between trabecular bone volume and tissue volume in tibia and femur, plus decreased connectivity density in femur and tibia (PubMed:23959653, PubMed:27119146). Osteoblast number is not affected, while osteoclast numbers are increased, suggesting that increased bone resorption is the cause for the low bone mass phenotype (PubMed:23959653). Lysine hydroxylation of skin and bone collagen alpha chains is strongly reduced (PubMed:27119146, PubMed:28115524). In contrast, prolyl 3-hydroxylation is not affected (PubMed:23959653, PubMed:27119146). Dorsal skin displays impaired packing of collagen fibrils, decreased skin tensile strength, decreased skin stiffness and increased skin fragility (PubMed:27119146, PubMed:28115524). Likewise, mice deficient for both P3h3 and P3h4 display decreased lysine hydroxylation of collagen alpha chains, but normal collagen prolyl 3-hydroxylation (PubMed:28115524).3 Publications

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 18Sequence analysisAdd BLAST18
ChainiPRO_000032029419 – 443Endoplasmic reticulum protein SC65Sequence analysisAdd BLAST425

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi367N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ8K2B0
PaxDbiQ8K2B0
PeptideAtlasiQ8K2B0
PRIDEiQ8K2B0

PTM databases

iPTMnetiQ8K2B0
PhosphoSitePlusiQ8K2B0

Expressioni

Tissue specificityi

Detected in calvaria, cartilage, kidney, ribs, and at lower levels in tail (PubMed:23959653, PubMed:27119146). Detected in proliferating and prehypertrophic chondrocytes in the growth plate of long bones and in mineralizing chondro-osseus bone collar and cortical bone (PubMed:23959653). Detected on osteoblasts in long bones (PubMed:23959653, PubMed:27119146). Detected in skin fibroblasts (at protein level) (PubMed:27119146). Detected in fetal ribs, and in tibia and metatarsus from neonates (PubMed:23959653).2 Publications

Gene expression databases

BgeeiENSMUSG00000006931 Expressed in 284 organ(s), highest expression level in vault of skull
CleanExiMM_1110036O03RIK
ExpressionAtlasiQ8K2B0 baseline and differential
GenevisibleiQ8K2B0 MM

Interactioni

Subunit structurei

Interacts with PLOD1, P3H3 and PPIB. Identified in a complex with PLOD1 and P3H3.1 Publication

Structurei

3D structure databases

ProteinModelPortaliQ8K2B0
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi352 – 443Asp/Glu-rich (acidic)CuratedAdd BLAST92

Sequence similaritiesi

Belongs to the leprecan family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiENOG410IQZF Eukaryota
ENOG41118PR LUCA
GeneTreeiENSGT00550000074573
HOGENOMiHOG000247068
HOVERGENiHBG005540
InParanoidiQ8K2B0
KOiK22461
OMAiYYRFYRE
OrthoDBiEOG091G06WO
TreeFamiTF320837

Family and domain databases

InterProiView protein in InterPro
IPR033213 SC65
PANTHERiPTHR13986:SF4 PTHR13986:SF4, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

Q8K2B0-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MARGAWGLLW LLLGSAGAQY EKYSFRGFPP EDLMPLATAY GHALEQYEGE
60 70 80 90 100
SWRESARYLE AALRLHRLLR DSEAFCHANC SGPATSQPRP ASGPDGDHDG
110 120 130 140 150
DGEDWARELR LFGHVLERAA CLRRCKKTLP AFQVPYPPRQ LLRDFQSRLP
160 170 180 190 200
YQYLHYAHFK ANRLEKAVAA AYTFLQRNPK HELTAKYLNY YRGMLDIGDE
210 220 230 240 250
SLTDLEAQPY EAVFLRAVKL YNSGDFRSST EDMERALAEY MTVFARCLAG
260 270 280 290 300
CEGAHEQVDF KDFYPAIADL FAESLQCKVD CETNLTPNVG GFFVDKFVAT
310 320 330 340 350
MYHYLQFAYY KLNDVRQAAR SAASYMLFDP KDSVMQQNLV YYRFHRARWG
360 370 380 390 400
LEDEDFQPRE EAMLYHNQTS ELRELLDFTH MYLQSDDEME LEETESLPEP
410 420 430 440
EEPLSDAEFE GEGDYEEGLY ADWWQEPEAK GDEAEAEPEP ELT
Length:443
Mass (Da):51,132
Last modified:October 1, 2002 - v1
Checksum:i99FA2B03344F15B1
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H3BK18H3BK18_MOUSE
Endoplasmic reticulum protein SC65
P3h4 Leprel4
103Annotation score:

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AL590968 Genomic DNA No translation available.
BC031856 mRNA Translation: AAH31856.1
CCDSiCCDS25421.1
RefSeqiNP_789800.1, NM_176830.2
UniGeneiMm.4940

Genome annotation databases

EnsembliENSMUST00000066489; ENSMUSP00000065278; ENSMUSG00000006931
GeneIDi66180
KEGGimmu:66180
UCSCiuc007lla.1 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AL590968 Genomic DNA No translation available.
BC031856 mRNA Translation: AAH31856.1
CCDSiCCDS25421.1
RefSeqiNP_789800.1, NM_176830.2
UniGeneiMm.4940

3D structure databases

ProteinModelPortaliQ8K2B0
ModBaseiSearch...
MobiDBiSearch...

PTM databases

iPTMnetiQ8K2B0
PhosphoSitePlusiQ8K2B0

Proteomic databases

MaxQBiQ8K2B0
PaxDbiQ8K2B0
PeptideAtlasiQ8K2B0
PRIDEiQ8K2B0

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000066489; ENSMUSP00000065278; ENSMUSG00000006931
GeneIDi66180
KEGGimmu:66180
UCSCiuc007lla.1 mouse

Organism-specific databases

CTDi10609
MGIiMGI:1913430 P3h4

Phylogenomic databases

eggNOGiENOG410IQZF Eukaryota
ENOG41118PR LUCA
GeneTreeiENSGT00550000074573
HOGENOMiHOG000247068
HOVERGENiHBG005540
InParanoidiQ8K2B0
KOiK22461
OMAiYYRFYRE
OrthoDBiEOG091G06WO
TreeFamiTF320837

Miscellaneous databases

PROiPR:Q8K2B0
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000006931 Expressed in 284 organ(s), highest expression level in vault of skull
CleanExiMM_1110036O03RIK
ExpressionAtlasiQ8K2B0 baseline and differential
GenevisibleiQ8K2B0 MM

Family and domain databases

InterProiView protein in InterPro
IPR033213 SC65
PANTHERiPTHR13986:SF4 PTHR13986:SF4, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiSC65_MOUSE
AccessioniPrimary (citable) accession number: Q8K2B0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 26, 2008
Last sequence update: October 1, 2002
Last modified: November 7, 2018
This is version 102 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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