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Protein

Beta-klotho

Gene

KLB

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

Functioni

Contributes to the transcriptional repression of cholesterol 7-alpha-hydroxylase (CYP7A1), the rate-limiting enzyme in bile acid synthesis. Probably inactive as a glycosidase. Increases the ability of FGFR1 and FGFR4 to bind FGF21 (By similarity).By similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Enzyme and pathway databases

ReactomeiR-HSA-109704. PI3K Cascade.
R-HSA-1257604. PIP3 activates AKT signaling.
R-HSA-1307965. betaKlotho-mediated ligand binding.
R-HSA-2219530. Constitutive Signaling by Aberrant PI3K in Cancer.
R-HSA-5654228. Phospholipase C-mediated cascade, FGFR4.
R-HSA-5654712. FRS-mediated FGFR4 signaling.
R-HSA-5654719. SHC-mediated cascade:FGFR4.
R-HSA-5654720. PI-3K cascade:FGFR4.
R-HSA-5654733. Negative regulation of FGFR4 signaling.
R-HSA-5673001. RAF/MAP kinase cascade.
R-HSA-6811558. PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling.

Protein family/group databases

CAZyiGH1. Glycoside Hydrolase Family 1.
TCDBi8.A.49.1.2. the klotho auxiliary protein (klotho) family.

Names & Taxonomyi

Protein namesi
Recommended name:
Beta-klotho
Short name:
BKL
Short name:
BetaKlotho
Alternative name(s):
Klotho beta-like protein
Gene namesi
Name:KLB
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:15527. KLB.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 996996ExtracellularSequence analysisAdd
BLAST
Transmembranei997 – 101721HelicalSequence analysisAdd
BLAST
Topological domaini1018 – 104427CytoplasmicSequence analysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cell membrane, Membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA142671586.

Polymorphism and mutation databases

BioMutaiKLB.
DMDMi74750586.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 10441044Beta-klothoPRO_0000063905Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi71 – 711N-linked (GlcNAc...)Sequence analysis
Glycosylationi120 – 1201N-linked (GlcNAc...)Sequence analysis
Glycosylationi125 – 1251N-linked (GlcNAc...)Sequence analysis
Glycosylationi211 – 2111N-linked (GlcNAc...)Sequence analysis
Glycosylationi308 – 3081N-linked (GlcNAc...)Sequence analysis
Glycosylationi391 – 3911N-linked (GlcNAc...)Sequence analysis
Glycosylationi554 – 5541N-linked (GlcNAc...)Sequence analysis
Glycosylationi611 – 6111N-linked (GlcNAc...)Sequence analysis
Glycosylationi702 – 7021N-linked (GlcNAc...)Sequence analysis
Glycosylationi706 – 7061N-linked (GlcNAc...)Sequence analysis
Glycosylationi971 – 9711N-linked (GlcNAc...)Sequence analysis

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ86Z14.
PaxDbiQ86Z14.
PeptideAtlasiQ86Z14.
PRIDEiQ86Z14.

PTM databases

iPTMnetiQ86Z14.
PhosphoSiteiQ86Z14.

Expressioni

Gene expression databases

BgeeiENSG00000134962.
CleanExiHS_KLB.
GenevisibleiQ86Z14. HS.

Organism-specific databases

HPAiHPA021136.

Interactioni

Subunit structurei

Interacts with FGFR1 and FGFR4 (By similarity). Interacts with FGF19; this interaction is direct. Interacts (via C-terminus) with FGF21; this interaction is direct.By similarity2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
FGF21Q9NSA12EBI-8515198,EBI-3909329

GO - Molecular functioni

  • fibroblast growth factor binding Source: UniProtKB
  • fibroblast growth factor receptor binding Source: UniProtKB

Protein-protein interaction databases

IntActiQ86Z14. 2 interactions.
MINTiMINT-6799890.
STRINGi9606.ENSP00000257408.

Structurei

3D structure databases

ProteinModelPortaliQ86Z14.
SMRiQ86Z14. Positions 81-508, 521-967.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni77 – 508432Glycosyl hydrolase-1 1Add
BLAST
Regioni517 – 967451Glycosyl hydrolase-1 2Add
BLAST

Domaini

Sequence similaritiesi

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0626. Eukaryota.
COG2723. LUCA.
GeneTreeiENSGT00550000074452.
HOGENOMiHOG000060126.
HOVERGENiHBG081856.
InParanoidiQ86Z14.
OMAiMLARMKV.
OrthoDBiEOG091G0035.
PhylomeDBiQ86Z14.
TreeFamiTF314803.

Family and domain databases

Gene3Di3.20.20.80. 2 hits.
InterProiIPR001360. Glyco_hydro_1.
IPR013781. Glyco_hydro_catalytic_dom.
IPR017853. Glycoside_hydrolase_SF.
[Graphical view]
PANTHERiPTHR10353. PTHR10353. 1 hit.
PfamiPF00232. Glyco_hydro_1. 3 hits.
[Graphical view]
PRINTSiPR00131. GLHYDRLASE1.
SUPFAMiSSF51445. SSF51445. 2 hits.

Sequencei

Sequence statusi: Complete.

Q86Z14-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKPGCAAGSP GNEWIFFSTD EITTRYRNTM SNGGLQRSVI LSALILLRAV
60 70 80 90 100
TGFSGDGRAI WSKNPNFTPV NESQLFLYDT FPKNFFWGIG TGALQVEGSW
110 120 130 140 150
KKDGKGPSIW DHFIHTHLKN VSSTNGSSDS YIFLEKDLSA LDFIGVSFYQ
160 170 180 190 200
FSISWPRLFP DGIVTVANAK GLQYYSTLLD ALVLRNIEPI VTLYHWDLPL
210 220 230 240 250
ALQEKYGGWK NDTIIDIFND YATYCFQMFG DRVKYWITIH NPYLVAWHGY
260 270 280 290 300
GTGMHAPGEK GNLAAVYTVG HNLIKAHSKV WHNYNTHFRP HQKGWLSITL
310 320 330 340 350
GSHWIEPNRS ENTMDIFKCQ QSMVSVLGWF ANPIHGDGDY PEGMRKKLFS
360 370 380 390 400
VLPIFSEAEK HEMRGTADFF AFSFGPNNFK PLNTMAKMGQ NVSLNLREAL
410 420 430 440 450
NWIKLEYNNP RILIAENGWF TDSRVKTEDT TAIYMMKNFL SQVLQAIRLD
460 470 480 490 500
EIRVFGYTAW SLLDGFEWQD AYTIRRGLFY VDFNSKQKER KPKSSAHYYK
510 520 530 540 550
QIIRENGFSL KESTPDVQGQ FPCDFSWGVT ESVLKPESVA SSPQFSDPHL
560 570 580 590 600
YVWNATGNRL LHRVEGVRLK TRPAQCTDFV NIKKQLEMLA RMKVTHYRFA
610 620 630 640 650
LDWASVLPTG NLSAVNRQAL RYYRCVVSEG LKLGISAMVT LYYPTHAHLG
660 670 680 690 700
LPEPLLHADG WLNPSTAEAF QAYAGLCFQE LGDLVKLWIT INEPNRLSDI
710 720 730 740 750
YNRSGNDTYG AAHNLLVAHA LAWRLYDRQF RPSQRGAVSL SLHADWAEPA
760 770 780 790 800
NPYADSHWRA AERFLQFEIA WFAEPLFKTG DYPAAMREYI ASKHRRGLSS
810 820 830 840 850
SALPRLTEAE RRLLKGTVDF CALNHFTTRF VMHEQLAGSR YDSDRDIQFL
860 870 880 890 900
QDITRLSSPT RLAVIPWGVR KLLRWVRRNY GDMDIYITAS GIDDQALEDD
910 920 930 940 950
RLRKYYLGKY LQEVLKAYLI DKVRIKGYYA FKLAEEKSKP RFGFFTSDFK
960 970 980 990 1000
AKSSIQFYNK VISSRGFPFE NSSSRCSQTQ ENTECTVCLF LVQKKPLIFL
1010 1020 1030 1040
GCCFFSTLVL LLSIAIFQRQ KRRKFWKAKN LQHIPLKKGK RVVS
Length:1,044
Mass (Da):119,808
Last modified:June 1, 2003 - v1
Checksum:i6EA0198250F8928F
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti65 – 651P → A.
Corresponds to variant rs34905034 [ dbSNP | Ensembl ].
VAR_061207
Natural varianti728 – 7281R → Q.
Corresponds to variant rs17618244 [ dbSNP | Ensembl ].
VAR_034053
Natural varianti747 – 7471A → V.
Corresponds to variant rs35372803 [ dbSNP | Ensembl ].
VAR_034054
Natural varianti906 – 9061Y → H.
Corresponds to variant rs17618262 [ dbSNP | Ensembl ].
VAR_049296
Natural varianti1020 – 10201Q → K.
Corresponds to variant rs4975017 [ dbSNP | Ensembl ].
VAR_034055

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB079373 mRNA. Translation: BAC56857.1.
BC104871 mRNA. Translation: AAI04872.1.
BC113653 mRNA. Translation: AAI13654.1.
CCDSiCCDS3451.1.
RefSeqiNP_783864.1. NM_175737.3.
UniGeneiHs.90756.

Genome annotation databases

EnsembliENST00000257408; ENSP00000257408; ENSG00000134962.
GeneIDi152831.
KEGGihsa:152831.
UCSCiuc003gua.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB079373 mRNA. Translation: BAC56857.1.
BC104871 mRNA. Translation: AAI04872.1.
BC113653 mRNA. Translation: AAI13654.1.
CCDSiCCDS3451.1.
RefSeqiNP_783864.1. NM_175737.3.
UniGeneiHs.90756.

3D structure databases

ProteinModelPortaliQ86Z14.
SMRiQ86Z14. Positions 81-508, 521-967.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ86Z14. 2 interactions.
MINTiMINT-6799890.
STRINGi9606.ENSP00000257408.

Protein family/group databases

CAZyiGH1. Glycoside Hydrolase Family 1.
TCDBi8.A.49.1.2. the klotho auxiliary protein (klotho) family.

PTM databases

iPTMnetiQ86Z14.
PhosphoSiteiQ86Z14.

Polymorphism and mutation databases

BioMutaiKLB.
DMDMi74750586.

Proteomic databases

MaxQBiQ86Z14.
PaxDbiQ86Z14.
PeptideAtlasiQ86Z14.
PRIDEiQ86Z14.

Protocols and materials databases

DNASUi152831.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000257408; ENSP00000257408; ENSG00000134962.
GeneIDi152831.
KEGGihsa:152831.
UCSCiuc003gua.4. human.

Organism-specific databases

CTDi152831.
GeneCardsiKLB.
H-InvDBHIX0031711.
HGNCiHGNC:15527. KLB.
HPAiHPA021136.
MIMi611135. gene.
neXtProtiNX_Q86Z14.
PharmGKBiPA142671586.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0626. Eukaryota.
COG2723. LUCA.
GeneTreeiENSGT00550000074452.
HOGENOMiHOG000060126.
HOVERGENiHBG081856.
InParanoidiQ86Z14.
OMAiMLARMKV.
OrthoDBiEOG091G0035.
PhylomeDBiQ86Z14.
TreeFamiTF314803.

Enzyme and pathway databases

ReactomeiR-HSA-109704. PI3K Cascade.
R-HSA-1257604. PIP3 activates AKT signaling.
R-HSA-1307965. betaKlotho-mediated ligand binding.
R-HSA-2219530. Constitutive Signaling by Aberrant PI3K in Cancer.
R-HSA-5654228. Phospholipase C-mediated cascade, FGFR4.
R-HSA-5654712. FRS-mediated FGFR4 signaling.
R-HSA-5654719. SHC-mediated cascade:FGFR4.
R-HSA-5654720. PI-3K cascade:FGFR4.
R-HSA-5654733. Negative regulation of FGFR4 signaling.
R-HSA-5673001. RAF/MAP kinase cascade.
R-HSA-6811558. PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling.

Miscellaneous databases

GenomeRNAii152831.
PROiQ86Z14.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000134962.
CleanExiHS_KLB.
GenevisibleiQ86Z14. HS.

Family and domain databases

Gene3Di3.20.20.80. 2 hits.
InterProiIPR001360. Glyco_hydro_1.
IPR013781. Glyco_hydro_catalytic_dom.
IPR017853. Glycoside_hydrolase_SF.
[Graphical view]
PANTHERiPTHR10353. PTHR10353. 1 hit.
PfamiPF00232. Glyco_hydro_1. 3 hits.
[Graphical view]
PRINTSiPR00131. GLHYDRLASE1.
SUPFAMiSSF51445. SSF51445. 2 hits.
ProtoNetiSearch...

Entry informationi

Entry nameiKLOTB_HUMAN
AccessioniPrimary (citable) accession number: Q86Z14
Secondary accession number(s): Q2M3K8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 11, 2005
Last sequence update: June 1, 2003
Last modified: September 7, 2016
This is version 116 of the entry and version 1 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, Reference proteome

Documents

  1. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
  2. Human chromosome 4
    Human chromosome 4: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  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.