Lipopolysaccharide-responsive and beige-like anchor protein Recombinant Protein | LRBA recombinant protein

Recombinant Human Lipopolysaccharide-responsive and beige-like anchor protein

Cat. Num. AAA969559

• Gene Names: LRBA; BGL; LBA; CDC4L; CVID8; LAB300
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Lipopolysaccharide-responsive and beige-like anchor protein; Recombinant Human Lipopolysaccharide-responsive and beige-like anchor protein; Beige-like protein; CDC4-like protein; LRBA recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Positions: 1267-1500aa; Partial
• Sequence: PQPHRHVLEISRQHEQPGQGIAPDAVNGQRRDSRSTVFRIPEFNWSQMHQRLLTDLLFSIETDIQMWRSHSTKTVMDFVNSSDNVIFVHNTIHLISQVMDNMVMACGGILPLLSAATSATHELENIEPTQGLSIEASVTFLQRLISLVDVLIFASSLGFTEIEAEKSMSSGGILRQCLRLVCAVAVRNCLECQQHSQLKTRGDKALKPMHSLIPLGKSAAKSPVDIVTGGISPV
• Sequence Length: 2851

• Preparation and Storage: Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

SDS-PAGE

Related Product Information for LRBA recombinant protein

May be involved in coupling signal transduction and vesicle trafficking to enable polarized secretion and/or membrane deposition of immune effector molecules.

Product Categories/Family for LRBA recombinant protein

Signal Transduction

References

Identification of a novel lipopolysaccharide-inducible gene with key features of both A kinase anchor proteins and chs1/beige proteins.Wang J.-W., Howson J., Haller E., Kerr W.G.J. Immunol. 166:4586-4595(2001) BCL8 is a novel, evolutionarily conserved human gene family encoding proteins with presumptive protein kinase A anchoring function.Dyomin V.G., Chaganti S.R., Dyomina K., Palanisamy N., Murty V.V.V.S., Dalla-Favera R., Chaganti R.S.K.Genomics 80:158-165(2002) Generation and annotation of the DNA sequences of human chromosomes 2 and 4.Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H., Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.Nature 434:724-731(2005) Mager D.L.Strategy for detecting cellular transcripts promoted by human endogenous long terminal repeats identification of a novel gene (CDC4L) with homology to yeast CDC4.Feuchter A.E., Freeman J.D., Mager D.L.Genomics 13:1237-1246(1992) Global, in vivo, and site-specific phosphorylation dynamics in signaling networks.Olsen J.V., Blagoev B., Gnad F., Macek B., Kumar C., Mortensen P., Mann M.Cell 127:635-648(2006) ATM and ATR substrate analysis reveals extensive protein networks responsive to DNA damage.Matsuoka S., Ballif B.A., Smogorzewska A., McDonald E.R. III, Hurov K.E., Luo J., Bakalarski C.E., Zhao Z., Solimini N., Lerenthal Y., Shiloh Y., Gygi S.P., Elledge S.J.Science 316:1160-1166(2007) A quantitative atlas of mitotic phosphorylation.Dephoure N., Zhou C., Villen J., Beausoleil S.A., Bakalarski C.E., Elledge S.J., Gygi S.P.Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.Gauci S., Helbig A.O., Slijper M., Krijgsveld J., Heck A.J., Mohammed S.Anal. Chem. 81:4493-4501(2009) Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions.Mayya V., Lundgren D.H., Hwang S.-I., Rezaul K., Wu L., Eng J.K., Rodionov V., Han D.K.Sci. Signal. 2:RA46-RA46(2009) Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis.Olsen J.V., Vermeulen M., Santamaria A., Kumar C., Miller M.L., Jensen L.J., Gnad F., Cox J., Jensen T.S., Nigg E.A., Brunak S., Mann M.Sci. Signal. 3:RA3-RA3(2010) Initial characterization of the human central proteome.Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.BMC Syst. Biol. 5:17-17(2011) System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation.Rigbolt K.T., Prokhorova T.A., Akimov V., Henningsen J., Johansen P.T., Kratchmarova I., Kassem M., Mann M., Olsen J.V., Blagoev B.Sci. Signal. 4:RS3-RS3(2011) N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB.Van Damme P., Lasa M., Polevoda B., Gazquez C., Elosegui-Artola A., Kim D.S., De Juan-Pardo E., Demeyer K., Hole K., Larrea E., Timmerman E., Prieto J., Arnesen T., Sherman F., Gevaert K., Aldabe R.Proc. Natl. Acad. Sci. U.S.A. 109:12449-12454(2012) An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.J. Proteomics 96:253-262(2014) Crystal structure of the PH-BEACH domains of human LRBA/BGL.Gebauer D., Li J., Jogl G., Shen Y., Myszka D.G., Tong L.Biochemistry 43:14873-14880(2004) The consensus coding sequences of human breast and colorectal cancers.Sjoeblom T., Jones S., Wood L.D., Parsons D.W., Lin J., Barber T.D., Mandelker D., Leary R.J., Ptak J., Silliman N., Szabo S., Buckhaults P., Farrell C., Meeh P., Markowitz S.D., Willis J., Dawson D., Willson J.K.V., Gazdar A.F., Hartigan J., Wu L., Liu C., Parmigiani G., Park B.H., Bachman K.E., Papadopoulos N., Vogelstein B., Kinzler K.W., Velculescu V.E.Science 314:268-274(2006) Deleterious mutations in LRBA are associated with a syndrome of immune deficiency and autoimmunity.Lopez-Herrera G., Tampella G., Pan-Hammarstrom Q., Herholz P., Trujillo-Vargas C.M., Phadwal K., Simon A.K., Moutschen M., Etzioni A., Mory A., Srugo I., Melamed D., Hultenby K., Liu C., Baronio M., Vitali M., Philippet P., Dideberg V., Aghamohammadi A., Rezaei N., Enright V., Du L., Salzer U., Eibel H., Pfeifer D., Veelken H., Stauss H., Lougaris V., Plebani A., Gertz E.M., Schaffer A.A., Hammarstrom L., Grimbacher B.Am. 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NCBI and Uniprot Product Information

• NCBI GI #: 345498499
• NCBI GeneID: 987
• NCBI Accession #: NP_001186211.2
• NCBI GenBank Nucleotide #: NM_001199282.2
• UniProt Accession #: P50851; Q4W5J4; Q4W5L6; Q8NFQ0; Q9H2U3; Q9H2U4
• Molecular Weight: 29.8 kDa
• NCBI Official Full Name: lipopolysaccharide-responsive and beige-like anchor protein isoform 1
• NCBI Official Synonym Full Names: LPS-responsive vesicle trafficking, beach and anchor containing
• NCBI Official Symbol: LRBA
• NCBI Official Synonym Symbols: BGL; LBA; CDC4L; CVID8; LAB300
• NCBI Protein Information: lipopolysaccharide-responsive and beige-like anchor protein
• UniProt Protein Name: Lipopolysaccharide-responsive and beige-like anchor protein
• Protein Family: Lipopolysaccharide-responsive and beige-like anchor protein
• UniProt Gene Name: LRBA
• UniProt Synonym Gene Names: BGL; CDC4L; LBA
• UniProt Entry Name: LRBA_HUMAN

NCBI Description

The protein encoded by this gene is a member of the WDL-BEACH-WD (WBW) gene family. Its expression is induced in B cells and macrophages by bacterial lipopolysaccharides (LPS). The encoded protein associates with protein kinase A and may be involved in leading intracellular vesicles to activated receptor complexes, which aids in the secretion and/or membrane deposition of immune effector molecules. Defects in this gene are associated with the disorder common variable immunodeficiency-8 with autoimmunity. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2012]

Uniprot Description

LRBA: May be involved in coupling signal transduction and vesicle trafficking to enable polarized secretion and/or membrane deposition of immune effector molecules. Defects in LRBA are the cause of immunodeficiency, common variable, type 8, with autoimmunity (CVID8). 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. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral

Chromosomal Location of Human Ortholog: 4q31.3

Cellular Component: cytoplasmic membrane-bound vesicle; endoplasmic reticulum; Golgi apparatus; integral to membrane; lysosome; membrane; plasma membrane

Disease: Immunodeficiency, Common Variable, 8, With Autoimmunity