HPA023597

antibody from Atlas Antibodies

Targeting: LRBA BGL, CDC4L, LAB300, LBA

Immunohistochemistry (Data presented on provider website)

Antibody Data

Product number

HPA023597

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.

References

Submitted references

BEACH domain proteins function as cargo-sorting adaptors in secretory and endocytic pathways

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)

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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