Antibody data
- Antibody Data
- Antigen structure
- References [13]
- Comments [0]
- Validations [0]
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Validation data
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- Product number
- HPA023597 - Provider product page
- Provider
- Atlas Antibodies
- Proper citation
- Atlas Antibodies Cat#HPA023597, RRID:AB_1853256
- Product name
- Anti-LRBA
- Antibody type
- Polyclonal
- Description
- Polyclonal Antibody against Human LRBA, Gene description: LPS-responsive vesicle trafficking, beach and anchor containing, Alternative Gene Names: BGL, CDC4L, LAB300, LBA, Validated applications: IHC, Uniprot ID: P50851, Storage: Store at +4°C for short term storage. Long time storage is recommended at -20°C.
- Reactivity
- Human
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 100 µl
- Concentration
- 0.3 mg/ml
- Storage
- Store at +4°C for short term storage. Long time storage is recommended at -20°C.
- Handling
- The antibody solution should be gently mixed before use.
Submitted references BEACH domain proteins function as cargo-sorting adaptors in secretory and endocytic pathways
Potential protein–phenotype correlation in three lipopolysaccharide-responsive beige-like anchor protein-deficient patients
Case Report: Infantile-Onset Fulminant Type 1 Diabetes Mellitus Caused by Novel Compound Heterozygous LRBA Variants
Clinical, immunological and genetic characteristic of patients with clinical phenotype associated to LRBA-deficiency in Colombia.
A Spectrum of Clinical Findings from ALPS to CVID: Several Novel LRBA Defects
Hijacking antibody-induced CTLA-4 lysosomal degradation for safer and more effective cancer immunotherapy
Rapid Flow Cytometry-Based Test for the Diagnosis of Lipopolysaccharide Responsive Beige-Like Anchor (LRBA) Deficiency
Diagnostic Yield of Next-generation Sequencing in Very Early-onset Inflammatory Bowel Diseases: A Multicentre Study
Treatment of Infantile Inflammatory Bowel Disease and Autoimmunity by Allogeneic Stem Cell Transplantation in LPS-Responsive Beige-Like Anchor Deficiency
Multiple Presentations of LRBA Deficiency: a Single-Center Experience
Atypical Manifestation of LPS-Responsive Beige-Like Anchor Deficiency Syndrome as an Autoimmune Endocrine Disorder without Enteropathy and Immunodeficiency
Deficiency of base excision repair enzyme NEIL3 drives increased predisposition to autoimmunity
Deleterious Mutations in LRBA Are Associated with a Syndrome of Immune Deficiency and Autoimmunity
Pankiv S, Dahl A, Aas A, Andersen R, Brech A, Holland P, Singh S, Bindesbøll C, Simonsen A
Journal of Cell Biology 2024;223(12)
Journal of Cell Biology 2024;223(12)
Potential protein–phenotype correlation in three lipopolysaccharide-responsive beige-like anchor protein-deficient patients
Tang W, Hu W, Huang Y, Wu B, Peng X, Zhai X, Qian X, Ye Z, Xia H, Wu J, Shi J
World Journal of Clinical Cases 2021;9(21):5873-5888
World Journal of Clinical Cases 2021;9(21):5873-5888
Case Report: Infantile-Onset Fulminant Type 1 Diabetes Mellitus Caused by Novel Compound Heterozygous LRBA Variants
Totsune E, Nakano T, Moriya K, Sato D, Suzuki D, Miura A, Katayama S, Niizuma H, Kanno J, van Zelm M, Imai K, Kanegane H, Sasahara Y, Kure S
Frontiers in Immunology 2021;12
Frontiers in Immunology 2021;12
Clinical, immunological and genetic characteristic of patients with clinical phenotype associated to LRBA-deficiency in Colombia.
Martínez-Jaramillo C, Gutierrez-Hincapie S, Orrego Arango J, Vazuez-Duque G, Erazo-Garnica R, Franco J, Trujillo-Vargas C
Colombia Medica 2019;50(3):176-91
Colombia Medica 2019;50(3):176-91
A Spectrum of Clinical Findings from ALPS to CVID: Several Novel LRBA Defects
Cagdas D, Halaçlı S, Tan Ç, Lo B, Çetinkaya P, Esenboğa S, Karaatmaca B, Matthews H, Balcı-Hayta B, Arıkoğlu T, Ezgü F, Aladağ E, Saltık-Temizel İ, Demir H, Kuşkonmaz B, Okur V, Gümrük F, Göker H, Çetinkaya D, Boztuğ K, Lenardo M, Sanal Ö, Tezcan İ
Journal of Clinical Immunology 2019;39(7):726-738
Journal of Clinical Immunology 2019;39(7):726-738
Hijacking antibody-induced CTLA-4 lysosomal degradation for safer and more effective cancer immunotherapy
Zhang Y, Du X, Liu M, Tang F, Zhang P, Ai C, Fields J, Sundberg E, Latinovic O, Devenport M, Zheng P, Liu Y
Cell Research 2019;29(8):609-627
Cell Research 2019;29(8):609-627
Rapid Flow Cytometry-Based Test for the Diagnosis of Lipopolysaccharide Responsive Beige-Like Anchor (LRBA) Deficiency
Gámez-Díaz L, Sigmund E, Reiser V, Vach W, Jung S, Grimbacher B
Frontiers in Immunology 2018;9
Frontiers in Immunology 2018;9
Diagnostic Yield of Next-generation Sequencing in Very Early-onset Inflammatory Bowel Diseases: A Multicentre Study
Charbit-Henrion F, Parlato M, Hanein S, Duclaux-Loras R, Nowak J, Begue B, Rakotobe S, Bruneau J, Fourrage C, Alibeu O, Rieux-Laucat F, Lévy E, Stolzenberg M, Mazerolles F, Latour S, Lenoir C, Fischer A, Picard C, Aloi M, Dias J, Hariz M, Bourrier A, Breuer C, Breton A, Bronsky J, Buderus S, Cananzi M, Coopman S, Crémilleux C, Dabadie A, Dumant-Forest C, Gurkan O, Fabre A, Fischer A, Diaz M, Gonzalez-Lama Y, Goulet O, Guariso G, Gurcan N, Homan M, Hugot J, Jeziorski E, Karanika E, Lachaux A, Lewindon P, Lima R, Magro F, Major J, Malamut G, Mas E, Mattyus I, Mearin L, Melek J, Navas-Lopez V, Paerregaard A, Pelatan C, Pigneur B, Pais I, Rebeuh J, Romano C, Siala N, Strisciuglio C, Tempia-Caliera M, Tounian P, Turner D, Urbonas V, Willot S, Ruemmele F, Cerf-Bensussan N
Journal of Crohn's and Colitis 2018;12(9):1104-1112
Journal of Crohn's and Colitis 2018;12(9):1104-1112
Treatment of Infantile Inflammatory Bowel Disease and Autoimmunity by Allogeneic Stem Cell Transplantation in LPS-Responsive Beige-Like Anchor Deficiency
Bakhtiar S, Gámez-Díaz L, Jarisch A, Soerensen J, Grimbacher B, Belohradsky B, Keller K, Rietschel C, Klingebiel T, Koletzko S, Albert M, Bader P
Frontiers in Immunology 2017;8
Frontiers in Immunology 2017;8
Multiple Presentations of LRBA Deficiency: a Single-Center Experience
Kostel Bal S, Haskologlu S, Serwas N, Islamoglu C, Aytekin C, Kendirli T, Kuloglu Z, Yavuz G, Dalgic B, Siklar Z, Kansu A, Ensari A, Boztug K, Dogu F, Ikinciogullari A
Journal of Clinical Immunology 2017;37(8):790-800
Journal of Clinical Immunology 2017;37(8):790-800
Atypical Manifestation of LPS-Responsive Beige-Like Anchor Deficiency Syndrome as an Autoimmune Endocrine Disorder without Enteropathy and Immunodeficiency
Bakhtiar S, Ruemmele F, Charbit-Henrion F, Lévy E, Rieux-Laucat F, Cerf-Bensussan N, Bader P, Paetow U
Frontiers in Pediatrics 2016;4
Frontiers in Pediatrics 2016;4
Deficiency of base excision repair enzyme NEIL3 drives increased predisposition to autoimmunity
Massaad M, Zhou J, Tsuchimoto D, Chou J, Jabara H, Janssen E, Glauzy S, Olson B, Morbach H, Ohsumi T, Schmitz K, Kyriacos M, Kane J, Torisu K, Nakabeppu Y, Notarangelo L, Chouery E, Megarbane A, Kang P, Al-Idrissi E, Aldhekri H, Meffre E, Mizui M, Tsokos G, Manis J, Al-Herz W, Wallace S, Geha R
Journal of Clinical Investigation 2016;126(11):4219-4236
Journal of Clinical Investigation 2016;126(11):4219-4236
Deleterious Mutations in LRBA Are Associated with a Syndrome of Immune Deficiency and Autoimmunity
Lopez-Herrera G, Tampella G, Pan-Hammarström Q, Herholz P, Trujillo-Vargas C, Phadwal K, Simon A, 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, Schäffer A, Hammarström L, Grimbacher B
The American Journal of Human Genetics 2012;90(6):986-1001
The American Journal of Human Genetics 2012;90(6):986-1001
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