ITB4_HUMAN » Integrin beta-4

ITB4_HUMAN » Integrin beta-4
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
ITB4_HUMAN » Integrin beta-4 » GP150;
Hydrophobic Thickness 35.6 ± 2.4 Å
Tilt Angle 2 ± 0°
ΔGtransfer -35.8 kcal/mol
ΔGfold -24.7 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome
Topology Out
TM Segments 711-734 (708-741)
Pathways

Arrhythmogenic right ventricular cardiomyopathy (KEGG)

Cell-Cell communication (Reactome)

Dilated cardiomyopathy (KEGG)

ECM-receptor interaction (KEGG)

Extracellular matrix organization (Reactome)

Focal adhesion (KEGG)

Hypertrophic cardiomyopathy (KEGG)

PI3K-Akt signaling pathway (KEGG)

Regulation of actin cytoskeleton (KEGG)

Signal Transduction (Reactome)

PDB 3f7q (1126-1355), 3f7r (1126-1369), 2yrz (1518-1622), 4wtw (1527-1618), 4wtx (1642-1736), 3h6a (989-1107), 3fso (989-1107), 3fq4 (989-1107), 1qg3 (A/B=1126-1320), 3f7p (C/D/E=1126-1369), 4q58 (C/D=1126-1320)
OPM none
Complexes none
Interactions

CADM1, Complex: CADM1:ITB4, PubMed

CLCA2, Complex: CLCA2:ITB4, PubMed

COHA1, Complex: COHA1:ITB4, PubMed

DYST, Complex: ITB4:DYST, PubMed

EGFR, Complex: ITB4:EGFR, PubMed

ERBB2, Complex: ERBB2:ITB4, PubMed

ITA3, Complex: ITA3:ITB4, PubMed

ITA6, Complex: ITA6:FYN:ITB4, PubMed

ITA6, Complex: LAMA5:LAMC1:ITA6:LAMB2:ITB4, PubMed

MET, Complex: MET:ITB4, PubMed

SE1L1, Complex: SE1L1:ITB4

Domains

AA: 28-73, PDBID: 1TYE, Subunit F, Seq Identity:38%, Integrin plexin domain

AA: 125-370, PDBID: 1YUK, Subunit B, Seq Identity:46%, Integrin beta chain VWA domain

AA: 543-573, PDBID: 1JV2, Subunit B, Seq Identity:51%, EGF-like domain

AA: 626-710, PDBID: 1JV2, Subunit B, Seq Identity:27%, Integrin beta tail domain

AA: 979-1084, PDBID: 3FQ4, Subunit B, Seq Identity:100%, Calx-beta domain

AA: 1128-1208, PDBID: 1QG3, Subunit A, Seq Identity:100%, Fibronectin type III domain

AA: 1221-1310, PDBID: 1QG3, Subunit A, Seq Identity:100%, Fibronectin type III domain

AA: 1529-1612, PDBID: 2YRZ, Subunit A, Seq Identity:100%, Fibronectin type III domain

AA: 1642-1728, PDBID: 4WTX, Subunit A, Seq Identity:100%, Fibronectin type III domain

UniProt annotation for ITB4_HUMAN » Integrin beta-4
FUNCTION: Integrin alpha-6/beta-4 is a receptor for laminin. Plays a critical structural role in the hemidesmosome of epithelial cells. Is required for the regulation of keratinocyte polarity and motility.

SUBUNIT: Heterodimer of an alpha and a beta subunit. Beta-4 associates with alpha-6. Interacts (via cytoplasmic region) with COL17A1 (via cytoplasmic region). Interacts (via cytoplasmic region) with DST isoform 3 (via N-terminus). Isoform beta-4a interacts (via cytoplasmic domain) with DST (via N-terminus). Interacts with RAC1.

TISSUE SPECIFICITY: Integrin alpha-6/beta-4 is predominantly expressed by epithelia. Isoform beta-4D is also expressed in colon and placenta. Isoform beta-4E is also expressed in epidermis, lung, duodenum, heart, spleen and stomach.

DOMAIN: The fibronectin type-III-like domains bind BPAG1 and plectin and probably also recruit BP230.

DISEASE: Epidermolysis bullosa letalis, with pyloric atresia (EB- PA) OMIM: An autosomal recessive, frequently lethal, epidermolysis bullosa with variable involvement of skin, nails, mucosa, and with variable effects on the digestive system. It is characterized by mucocutaneous fragility, aplasia cutis congenita, and gastrointestinal atresia, which most commonly affects the pylorus. Pyloric atresia is a primary manifestation rather than a scarring process secondary to epidermolysis bullosa. mutations affecting the gene represented in this entry.

DISEASE: Generalized atrophic benign epidermolysis bullosa (GABEB) OMIM: A non-lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities. mutations affecting the gene represented in this entry.

UniProt features for ITB4_HUMAN » Integrin beta-4
SIGNAL 1 27
CHAIN 28 1822 Integrin beta-4.
DOMAIN 29 73 PSI.
DOMAIN 131 329 VWFA.
REPEAT 456 502 I.
REPEAT 503 542 II.
REPEAT 543 581 III.
REPEAT 582 619 IV.
DOMAIN 979 1084 Calx-beta.
DOMAIN 1126 1215 Fibronectin type-III 1.
DOMAIN 1220 1318 Fibronectin type-III 2.
DOMAIN 1528 1618 Fibronectin type-III 3.
DOMAIN 1641 1734 Fibronectin type-III 4.
REGION 456 619 Cysteine-rich tandem repeats.
DISULFID 30 455 By similarity.
DISULFID 38 48 By similarity.
DISULFID 41 72 By similarity.
DISULFID 51 61 By similarity.
DISULFID 245 288 By similarity.
DISULFID 424 671 By similarity.
DISULFID 452 457 By similarity.
DISULFID 468 479 By similarity.
DISULFID 476 512 By similarity.
DISULFID 481 490 By similarity.
DISULFID 492 503 By similarity.
DISULFID 518 523 By similarity.
DISULFID 520 551 By similarity.
DISULFID 525 536 By similarity.
DISULFID 557 562 By similarity.
DISULFID 564 573 By similarity.
DISULFID 575 582 By similarity.
DISULFID 596 601 By similarity.
DISULFID 598 648 By similarity.
DISULFID 603 614 By similarity.
DISULFID 626 635 By similarity.
DISULFID 632 706 By similarity.
DISULFID 651 680 By similarity.
Amino Acid Sequence for ITB4_HUMAN » Integrin beta-4
MAGPRPSPWA RLLLAALISV SLSGTLANRC KKAPVKSCTE CVRVDKDCAY CTDEMFRDRR CNTQAELLAA GCQRESIVVM ESSFQITEET QIDTTLRRSQ MSPQGLRVRL RPGEERHFEL EVFEPLESPV DLYILMDFSN SMSDDLDNLK KMGQNLARVL SQLTSDYTIG FGKFVDKVSV PQTDMRPEKL KEPWPNSDPP FSFKNVISLT EDVDEFRNKL QGERISGNLD APEGGFDAIL QTAVCTRDIG WRPDSTHLLV FSTESAFHYE ADGANVLAGI MSRNDERCHL DTTGTYTQYR TQDYPSVPTL VRLLAKHNII PIFAVTNYSY SYYEKLHTYF PVSSLGVLQE DSSNIVELLE EAFNRIRSNL DIRALDSPRG LRTEVTSKMF QKTRTGSFHI RRGEVGIYQV QLRALEHVDG THVCQLPEDQ KGNIHLKPSF SDGLKMDAGI ICDVCTCELQ KEVRSARCSF NGDFVCGQCV CSEGWSGQTC NCSTGSLSDI QPCLREGEDK PCSGRGECQC GHCVCYGEGR YEGQFCEYDN FQCPRTSGFL CNDRGRCSMG QCVCEPGWTG PSCDCPLSNA TCIDSNGGIC NGRGHCECGR CHCHQQSLYT DTICEINYSA IHPGLCEDLR SCVQCQAWGT GEKKGRTCEE CNFKVKMVDE LKRAEEVVVR CSFRDEDDDC TYSYTMEGDG APGPNSTVLV HKKKDCPPGS FWWLIPLLLL LLPLLALLLL LCWKYCACCK ACLALLPCCN RGHMVGFKED HYMLRENLMA SDHLDTPMLR SGNLKGRDVV RWKVTNNMQR PGFATHAASI NPTELVPYGL SLRLARLCTE NLLKPDTREC AQLRQEVEEN LNEVYRQISG VHKLQQTKFR QQPNAGKKQD HTIVDTVLMA PRSAKPALLK LTEKQVEQRA FHDLKVAPGY YTLTADQDAR GMVEFQEGVE LVDVRVPLFI RPEDDDEKQL LVEAIDVPAG TATLGRRLVN ITIIKEQARD VVSFEQPEFS VSRGDQVARI PVIRRVLDGG KSQVSYRTQD GTAQGNRDYI PVEGELLFQP GEAWKELQVK LLELQEVDSL LRGRQVRRFH VQLSNPKFGA HLGQPHSTTI IIRDPDELDR SFTSQMLSSQ PPPHGDLGAP QNPNAKAAGS RKIHFNWLPP SGKPMGYRVK YWIQGDSESE AHLLDSKVPS VELTNLYPYC DYEMKVCAYG AQGEGPYSSL VSCRTHQEVP SEPGRLAFNV VSSTVTQLSW AEPAETNGEI TAYEVCYGLV NDDNRPIGPM KKVLVDNPKN RMLLIENLRE SQPYRYTVKA RNGAGWGPER EAIINLATQP KRPMSIPIIP DIPIVDAQSG EDYDSFLMYS DDVLRSPSGS QRPSVSDDTG CGWKFEPLLG EELDLRRVTW RLPPELIPRL SASSGRSSDA EAPHGPPDDG GAGGKGGSLP RSATPGPPGE HLVNGRMDFA FPGSTNSLHR MTTTSAAAYG THLSPHVPHR VLSTSSTLTR DYNSLTRSEH SHSTTLPRDY STLTSVSSHD SRLTAGVPDT PTRLVFSALG PTSLRVSWQE PRCERPLQGY SVEYQLLNGG ELHRLNIPNP AQTSVVVEDL LPNHSYVFRV RAQSQEGWGR EREGVITIES QVHPQSPLCP LPGSAFTLST PSAPGPLVFT ALSPDSLQLS WERPRRPNGD IVGYLVTCEM AQGGGPATAF RVDGDSPESR LTVPGLSENV PYKFKVQART TEGFGPEREG IITIESQDGG PFPQLGSRAG LFQHPLQSEY SSITTTHTSA TEPFLVDGPT LGAQHLEAGG SLTRHVTQEF VSRTLTTSGT LSTHMDQQFF QT