Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Beta-klotho

Gene

KLB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

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

EuPathDBiHostDB:ENSG00000134962.6
HGNCiHGNC:15527 KLB
MIMi611135 gene
neXtProtiNX_Q86Z14

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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 996ExtracellularSequence analysisAdd BLAST996
Transmembranei997 – 1017HelicalSequence analysisAdd BLAST21
Topological domaini1018 – 1044CytoplasmicSequence analysisAdd BLAST27

Keywords - Cellular componenti

Cell membrane, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi152831
OpenTargetsiENSG00000134962
PharmGKBiPA142671586

Polymorphism and mutation databases

BioMutaiKLB
DMDMi74750586

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000639051 – 1044Beta-klothoAdd BLAST1044

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi71N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi120N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi125N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi211N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi308N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi391N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi554N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi611N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi702N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi706N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi971N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ86Z14
PaxDbiQ86Z14
PeptideAtlasiQ86Z14
PRIDEiQ86Z14

PTM databases

iPTMnetiQ86Z14
PhosphoSitePlusiQ86Z14

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
  • Ras guanyl-nucleotide exchange factor activity Source: Reactome

Protein-protein interaction databases

CORUMiQ86Z14
IntActiQ86Z14, 2 interactors
MINTiQ86Z14
STRINGi9606.ENSP00000257408

Structurei

Secondary structure

11044
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi56 – 61Combined sources6
Beta strandi86 – 90Combined sources5
Helixi93 – 96Combined sources4
Helixi102 – 104Combined sources3
Helixi109 – 117Combined sources9
Turni130 – 132Combined sources3
Helixi134 – 144Combined sources11
Beta strandi147 – 152Combined sources6
Helixi155 – 158Combined sources4
Beta strandi162 – 166Combined sources5
Helixi169 – 184Combined sources16
Beta strandi188 – 196Combined sources9
Helixi200 – 206Combined sources7
Helixi208 – 210Combined sources3
Helixi214 – 229Combined sources16
Turni230 – 232Combined sources3
Beta strandi235 – 240Combined sources6
Helixi242 – 250Combined sources9
Helixi263 – 287Combined sources25
Helixi289 – 292Combined sources4
Beta strandi295 – 301Combined sources7
Beta strandi304 – 306Combined sources3
Helixi313 – 326Combined sources14
Helixi328 – 335Combined sources8
Beta strandi336 – 339Combined sources4
Helixi342 – 347Combined sources6
Turni348 – 351Combined sources4
Helixi357 – 363Combined sources7
Beta strandi368 – 375Combined sources8
Helixi376 – 378Combined sources3
Helixi382 – 384Combined sources3
Beta strandi390 – 393Combined sources4
Helixi396 – 406Combined sources11
Beta strandi411 – 417Combined sources7
Helixi430 – 450Combined sources21
Beta strandi453 – 460Combined sources8
Helixi468 – 470Combined sources3
Beta strandi473 – 475Combined sources3
Beta strandi478 – 480Combined sources3
Beta strandi485 – 487Combined sources3
Beta strandi490 – 492Combined sources3
Helixi494 – 505Combined sources12
Beta strandi526 – 533Combined sources8
Helixi576 – 578Combined sources3
Helixi580 – 589Combined sources10
Beta strandi595 – 600Combined sources6
Helixi603 – 606Combined sources4
Helixi612 – 614Combined sources3
Helixi617 – 632Combined sources16
Beta strandi636 – 642Combined sources7
Helixi653 – 657Combined sources5
Helixi660 – 662Combined sources3
Helixi665 – 681Combined sources17
Turni682 – 684Combined sources3
Beta strandi687 – 692Combined sources6
Helixi694 – 696Combined sources3
Turni698 – 700Combined sources3
Helixi705 – 729Combined sources25
Helixi731 – 734Combined sources4
Beta strandi737 – 743Combined sources7
Beta strandi746 – 751Combined sources6
Helixi755 – 768Combined sources14
Helixi770 – 777Combined sources8
Helixi784 – 795Combined sources12
Helixi808 – 814Combined sources7
Beta strandi819 – 824Combined sources6
Beta strandi828 – 833Combined sources6
Beta strandi838 – 840Combined sources3
Helixi841 – 844Combined sources4
Beta strandi847 – 850Combined sources4
Helixi866 – 880Combined sources15
Beta strandi885 – 889Combined sources5
Helixi901 – 919Combined sources19
Beta strandi925 – 931Combined sources7
Helixi954 – 964Combined sources11

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5VAKX-ray1.70A30-522[»]
5VANX-ray2.20A30-983[»]
5VAQX-ray2.61A30-983[»]
ProteinModelPortaliQ86Z14
SMRiQ86Z14
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni77 – 508Glycosyl hydrolase-1 1Add BLAST432
Regioni517 – 967Glycosyl hydrolase-1 2Add BLAST451

Domaini

Sequence similaritiesi

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0626 Eukaryota
COG2723 LUCA
GeneTreeiENSGT00550000074452
HOGENOMiHOG000060126
HOVERGENiHBG081856
InParanoidiQ86Z14
KOiK22404
OMAiMLARMKV
OrthoDBiEOG091G0035
PhylomeDBiQ86Z14
TreeFamiTF314803

Family and domain databases

InterProiView protein in InterPro
IPR001360 Glyco_hydro_1
IPR017853 Glycoside_hydrolase_SF
PANTHERiPTHR10353 PTHR10353, 6 hits
PfamiView protein in Pfam
PF00232 Glyco_hydro_1, 3 hits
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)DescriptionActionsGraphical viewLength
Natural variantiVAR_06120765P → A. Corresponds to variant dbSNP:rs34905034Ensembl.1
Natural variantiVAR_034053728R → Q. Corresponds to variant dbSNP:rs17618244Ensembl.1
Natural variantiVAR_034054747A → V. Corresponds to variant dbSNP:rs35372803Ensembl.1
Natural variantiVAR_049296906Y → H. Corresponds to variant dbSNP:rs17618262Ensembl.1
Natural variantiVAR_0340551020Q → K. Corresponds to variant dbSNP:rs4975017Ensembl.1

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

Similar proteinsi

Entry informationi

Entry nameiKLOTB_HUMAN
AccessioniPrimary (citable) accession number: Q86Z14
Secondary accession number(s): Q2M3K8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 11, 2005
Last sequence update: June 1, 2003
Last modified: April 25, 2018
This is version 126 of the entry and version 1 of the sequence. See 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

3D-structure, 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

Cookie policy

We would like to use anonymized google analytics cookies to gather statistics on how uniprot.org is used in aggregate. Learn more

UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health