LRBA_HUMAN » Lipopolysaccharide-responsive and beige-like anchor protein

LRBA_HUMAN » Lipopolysaccharide-responsive and beige-like anchor protein
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Topology in Golgi apparatus membrane
TopologyGolgi lumenal side
cytoplasmic side
LRBA_HUMAN » Lipopolysaccharide-responsive and beige-like anchor protein » Beige-like protein;CDC4-like protein;
Hydrophobic Thickness 30.6 ± 3.1 Å
Tilt Angle 8 ± 5°
ΔGtransfer -18.7 kcal/mol
ΔGfold -14.9 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 1528-1548 (1523-1549)
Pathways none
PDB 1t77 (2076-2489)
OPM none
Complexes none
Interactions

NOTC1, Complex: LRBA:NOTC1, PubMed

Domains

AA: 211-377, PDBID: 4QVS, Subunit A, Seq Identity:13%, Concanavalin A-like lectin/glucanases superfamily

AA: 446-717, Domain of unknown function (DUF4704)

AA: 1880-2058, Domain of Unknown Function (DUF1088)

AA: 2084-2181, PDBID: 1T77, Subunit C, Seq Identity:100%, PH domain associated with Beige/BEACH

AA: 2213-2489, PDBID: 1T77, Subunit C, Seq Identity:100%, Beige/BEACH domain

UniProt annotation for LRBA_HUMAN » Lipopolysaccharide-responsive and beige-like anchor protein
FUNCTION: May be involved in coupling signal transduction and vesicle trafficking to enable polarized secretion and/or membrane deposition of immune effector molecules.

TISSUE SPECIFICITY: Ubiquitous.

INDUCTION: By bacterial lipopolysaccharides (LPS).

DISEASE: Immunodeficiency, common variable, 8, with autoimmunity (CVID8) OMIM: An autosomal recessive immunologic disorder associated with defective B-cell differentiation and decreased or absent antibody production. Affected individuals have early- childhood onset of recurrent infections, particularly respiratory infections, and also develop variable autoimmune disorders, including idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and inflammatory bowel disease. mutations affecting the gene represented in this entry.

UniProt features for LRBA_HUMAN » Lipopolysaccharide-responsive and beige-like anchor protein
CHAIN 2 2863 Lipopolysaccharide-responsive and beige- like anchor protein.
REPEAT 1301 1343 WD 1.
DOMAIN 2200 2489 BEACH.
REPEAT 2591 2633 WD 2.
REPEAT 2636 2679 WD 3.
REPEAT 2695 2735 WD 4.
REPEAT 2777 2816 WD 5.
REPEAT 2819 2858 WD 6.
COILED 1006 1053 Potential.
Amino Acid Sequence for LRBA_HUMAN » Lipopolysaccharide-responsive and beige-like anchor protein
MASEDNRVPS PPPTGDDGGG GGREETPTEG GALSLKPGLP IRGIRMKFAV LTGLVEVGEV SNRDIVETVF NLLVGGQFDL EMNFIIQEGE SINCMVDLLE KCDITCQAEV WSMFTAILKK SIRNLQVCTE VGLVEKVLGK IEKVDNMIAD LLVDMLGVLA SYNLTVRELK LFFSKLQGDK GRWPPHAGKL LSVLKHMPQK YGPDAFFNFP GKSAAAIALP PIAKWPYQNG FTFHTWLRMD PVNNINVDKD KPYLYCFRTS KGLGYSAHFV GGCLIVTSIK SKGKGFQHCV KFDFKPQKWY MVTIVHIYNR WKNSELRCYV NGELASYGEI TWFVNTSDTF DKCFLGSSET ADANRVFCGQ MTAVYLFSEA LNAAQIFAIY QLGLGYKGTF KFKAESDLFL AEHHKLLLYD GKLSSAIAFT YNPRATDAQL CLESSPKDNP SIFVHSPHAL MLQDVKAVLT HSIQSAMHSI GGVQVLFPLF AQLDYRQYLS DEIDLTICST LLAFIMELLK NSIAMQEQML ACKGFLVIGY SLEKSSKSHV SRAVLELCLA FSKYLSNLQN GMPLLKQLCD HVLLNPAIWI HTPAKVQLML YTYLSTEFIG TVNIYNTIRR VGTVLLIMHT LKYYYWAVNP QDRSGITPKG LDGPRPNQKE MLSLRAFLLM FIKQLVMKDS GVKEDELQAI LNYLLTMHED DNLMDVLQLL VALMSEHPNS MIPAFDQRNG LRVIYKLLAS KSEGIRVQAL KAMGYFLKHL APKRKAEVML GHGLFSLLAE RLMLQTNLIT MTTYNVLFEI LIEQIGTQVI HKQHPDPDSS VKIQNPQILK VIATLLRNSP QCPESMEVRR AFLSDMIKLF NNSRENRRSL LQCSVWQEWM LSLCYFNPKN SDEQKITEMV YAIFRILLYH AVKYEWGGWR VWVDTLSITH SKVTFEIHKE NLANIFREQQ GKVDEEIGLC SSTSVQAASG IRRDINVSVG SQQPDTKDSP VCPHFTTNGN ENSSIEKTSS LESASNIELQ TTNTSYEEMK AEQENQELPD EGTLEETLTN ETRNADDLEV SSDIIEAVAI SSNSFITTGK DSMTVSEVTA SISSPSEEDA SEMPEFLDKS IVEEEEDDDY VELKVEGSPT EEANLPTELQ DNSLSPAASE AGEKLDMFGN DDKLIFQEGK PVTEKQTDTE TQDSKDSGIQ TMTASGSSAM SPETTVSQIA VESDLGQMLE EGKKATNLTR ETKLINDCHG SVSEASSEQK IAKLDVSNVA TDTERLELKA SPNVEAPQPH RHVLEISRQH EQPGQGIAPD AVNGQRRDSR STVFRIPEFN WSQMHQRLLT DLLFSIETDI QMWRSHSTKT VMDFVNSSDN VIFVHNTIHL ISQVMDNMVM ACGGILPLLS AATSATHELE NIEPTQGLSI EASVTFLQRL ISLVDVLIFA SSLGFTEIEA EKSMSSGGIL RQCLRLVCAV AVRNCLECQQ HSQLKTRGDK ALKPMHSLIP LGKSAAKSPV DIVTGGISPV RDLDRLLQDM DINRLRAVVF RDIEDSKQAQ FLALAVVYFI SVLMVSKYRD ILEPQNERHS QSCTETGSEN ENVSLSEITP AAFSTLTTAS VEESESTSSA RRRDSGIGEE TATGLGSHVE VTPHTAPPGV SAGPDAISEV LSTLSLEVNK SPETKNDRGN DLDTKATPSV SVSKNVNVKD ILRSLVNIPA DGVTVDPALL PPACLGALGD LSVEQPVQFR SFDRSVIVAA KKSAVSPSTF NTSIPTNAVS VVSSVDSAQA SDMGGESPGS RSSNAKLPSV PTVDSVSQDP VSNMSITERL EHALEKAAPL LREIFVDFAP FLSRTLLGSH GQELLIEGTS LVCMKSSSSV VELVMLLCSQ EWQNSIQKNA GLAFIELVNE GRLLSQTMKD HLVRVANEAE FILSRQRAED IHRHAEFESL CAQYSADKRE DEKMCDHLIR AAKYRDHVTA TQLIQKIINI LTDKHGAWGN SAVSRPLEFW RLDYWEDDLR RRRRFVRNPL GSTHPEATLK TAVEHVCIFK LRENSKATDE DILAKGKQSI RSQALGNQNS ENEILLEGDD DTLSSVDEKD LENLAGPVSL STPAQLVAPS VVVKGTLSVT SSELYFEVDE EDPNFKKIDP KILAYTEGLH GKWLFTEIRS IFSRRYLLQN TALEIFMANR VAVMFNFPDP ATVKKVVNYL PRVGVGTSFG LPQTRRISLA SPRQLFKASN MTQRWQHREI SNFEYLMFLN TIAGRSYNDL NQYPVFPWVI TNYESEELDL TLPTNFRDLS KPIGALNPKR AAFFAERYES WEDDQVPKFH YGTHYSTASF VLAWLLRIEP FTTYFLNLQG GKFDHADRTF SSISRAWRNS QRDTSDIKEL IPEFYYLPEM FVNFNNYNLG VMDDGTVVSD VELPPWAKTS EEFVHINRLA LESEFVSCQL HQWIDLIFGY KQQGPEAVRA LNVFYYLTYE GAVNLNSITD PVLREAVEAQ IRSFGQTPSQ LLIEPHPPRG SAMQVSPLMF TDKAQQDVIM VLKFPSNSPV THVAANTQPG LATPAVITVT ANRLFAVNKW HNLPAHQGAV QDQPYQLPVE IDPLIASNTG MHRRQITDLL DQSIQVHSQC FVITSDNRYI LVCGFWDKSF RVYSTDTGRL IQVVFGHWDV VTCLARSESY IGGNCYILSG SRDATLLLWY WNGKCSGIGD NPGSETAAPR AILTGHDYEV TCAAVCAELG LVLSGSQEGP CLIHSMNGDL LRTLEGPENC LKPKLIQASR EGHCVIFYEN GLFCTFSVNG KLQATMETDD NIRAIQLSRD GQYLLTGGDR GVVVVRQVSD LKQLFAYPGC DAGIRAMALS YDQRCIISGM ASGSIVLFYN DFNRWHHEYQ TRY