Mouse Anti-BTD Recombinant Antibody (3B10-2B3) (CBMAB-A0897-LY)

Name: Mouse Anti-BTD Recombinant Antibody (3B10-2B3)
SKU: CBMAB-A0897-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

3B10-2B3

Application

WB, E

Immunogen

BTD (AAH12099, 1 a.a. ~ 543 a.a) full-length recombinant protein with GST tag.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG1, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS, pH 7.4

Preservative

None

Concentration

Batch dependent

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

biotinidase

Introduction

Biotinidase functions to recycle biotin in the body by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. Biotinidase has also been shown to have biotinyl-transferase activity. Defects in the biotinidase gene cause multiple carboxylase deficiency. [provided by RefSeq]

Entrez Gene ID

686

UniProt ID

P43251

Function

Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.

Biological Process

Biotin metabolic process Source: GO_Central
Central nervous system development Source: ProtInc

Cellular Location

Extracellular space

Involvement in disease

Biotinidase deficiency (BTD deficiency): A juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis. Biotinidase deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur.

Karaoglan, M., Nacarkahya, G., Keskin, M., & Keskin, O. (2021). Immunophenotypic analysis of lymphocyte subsets in newborns with biotinidase deficiency. Pediatric Allergy and Immunology, 32(3), 586-598.

Geng, J., Sun, Y., Zhao, Y., Xiong, W., Zhong, M., Zhang, Y., ... & Yuan, H. (2021). Two novel BTD mutations causing profound biotinidase deficiency in a Chinese patient. Molecular Genetics & Genomic Medicine, 9(2), e1591.

Oz, O., Karaca, M., Atas, N., Gonel, A., & Ercan, M. (2021). BTD Gene Mutations in Biotinidase Deficiency: Genotype-Phenotype Correlation. Journal of the College of Physicians and Surgeons--Pakistan: JCPSP, 30(7), 780-785.

Funghini, S., Tonin, R., Malvagia, S., Caciotti, A., Donati, M. A., Morrone, A., & la Marca, G. (2020). High frequency of biotinidase deficiency in Italian population identified by newborn screening. Molecular Genetics and Metabolism Reports, 25, 100689.

Canda, E., Uçar, S. K., & Çoker, M. (2020). Biotinidase Deficiency: Prevalence, Impact And Management Strategies. Pediatric Health, Medicine and Therapeutics, 11, 127.

Borsatto, T., Sperb-Ludwig, F., Blom, H. J., & Schwartz, I. V. (2019). Effect of BTD gene variants on in vitro biotinidase activity. Molecular genetics and metabolism, 127(4), 361-367.

Hsu, R. H., Chien, Y. H., Hwu, W. L., Chang, I. F., Ho, H. C., Chou, S. P., ... & Lee, N. C. (2019). Genotypic and phenotypic correlations of biotinidase deficiency in the Chinese population. Orphanet journal of rare diseases, 14(1), 1-6.

Liu, Z., Zhao, X., Sheng, H., Cai, Y., Yin, X., Chen, X., ... & Liu, L. (2018). Clinical features, BTD gene mutations, and their functional studies of eight symptomatic patients with biotinidase deficiency from Southern China. American journal of medical genetics Part A, 176(3), 589-596.

Murry, J. B., Machini, K., Ceyhan-Birsoy, O., Kritzer, A., Krier, J. B., Lebo, M. S., ... & Waisbren, S. E. (2018). Reconciling newborn screening and a novel splice variant in BTD associated with partial biotinidase deficiency: a BabySeq Project case report. Molecular Case Studies, 4(4), a002873.

Borsatto, T., Sperb-Ludwig, F., Lima, S. E., S. Carvalho, M. R., S. Fonseca, P. A., S. Camelo Jr, J., ... & D. Schwartz, I. V. (2017). Biotinidase deficiency: Genotype-biochemical phenotype association in Brazilian patients. PLoS One, 12(5), e0177503.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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