Mouse Anti-CYB5R3 Recombinant Antibody (CF128) (CBMAB-FT350LY)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CF128

Antibody Isotype

IgG2a

Application

ELISA, WB, IHC, IF

Basic Information

Immunogen

cytochrome b5 reductase 3

Specificity

Human

Antibody Isotype

IgG2a

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

50% glycerol

Preservative

0.02% sodium azide

Storage

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

Target

Full Name

Cytochrome B5 Reductase 3

Introduction

CYB5R3 (Cytochrome B5 Reductase 3) is a Protein Coding gene. Diseases associated with CYB5R3 include Methemoglobinemia Due To Deficiency Of Methemoglobin Reductase and Hereditary Methemoglobinemia. Among its related pathways are Innate Immune System and Metabolism. Gene Ontology (GO) annotations related to this gene include oxidoreductase activity and NAD binding. An important paralog of this gene is CYB5R1.

Entrez Gene ID

54205

UniProt ID

P00387

Alternative Names

DIA1

Function

Desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism, and, in erythrocyte, methemoglobin reduction.

Biological Process

Blood circulation Source: ProtInc
Cholesterol biosynthetic process Source: UniProtKB-KW
L-ascorbic acid metabolic process Source: Reactome
Neutrophil degranulation Source: Reactome
Nitric oxide biosynthetic process Source: FlyBase
Xenobiotic metabolic process Source: Reactome

Cellular Location

Isoform 1: Endoplasmic reticulum membrane; Mitochondrion outer membrane
Isoform 2: Cytoplasm. Produces the soluble form found in erythrocytes.

Involvement in disease

Methemoglobinemia CYB5R3-related (METHB-CYB5R3):
A form of methemoglobinemia, a hematologic disease characterized by the presence of excessive amounts of methemoglobin in blood cells, resulting in decreased oxygen carrying capacity of the blood, cyanosis and hypoxia. There are two types of methemoglobinemia CYB5R3-related. In type 1, the defect affects the soluble form of the enzyme, is restricted to red blood cells, and causes well-tolerated methemoglobinemia. In type 2, the defect affects both the soluble and microsomal forms of the enzyme and is thus generalized, affecting red cells, leukocytes and all body tissues. Type 2 methemoglobinemia is associated with mental deficiency and other neurologic symptoms.

More Infomation

References

Deorukhkar, A., Kulkarni, A., & Kedar, P. (2022). Three novel mutations in CYB5R3 gene causing NADH-cytochrome b5 reductase enzyme deficiency leads to recessive congenital methaemoglobinemia. Molecular Biology Reports, 1-7.

Wood, K. C., Durgin, B. G., Schmidt, H. M., Hahn, S. A., Baust, J. J., Bachman, T., ... & Straub, A. C. (2019). Smooth muscle cytochrome b5 reductase 3 deficiency accelerates pulmonary hypertension development in sickle cell mice. Blood advances, 3(23), 4104-4116.

Carew, N. T., Altmann, H. M., Galley, J. C., Hahn, S., Miller, M. P., Shiva, S., ... & Straub, A. C. (2018). Cytochrome b5 Reductase 3 Regulates Myoglobin Redox State and Controls Cardiac Function. Circulation Research, 123(Suppl_1), A103-A103.

Kedar, P. S., Gupta, V., Warang, P., Chiddarwar, A., & Madkaikar, M. (2018). Novel mutation (R192C) in CYB5R3 gene causing NADH-cytochrome b5 reductase deficiency in eight Indian patients associated with autosomal recessive congenital methemoglobinemia type-I. Hematology, 23(8), 567-573.

Carew, N. T., Altmann, H. M., Galley, J. C., Hahn, S., Miller, M. P., Shiva, S., ... & Straub, A. C. (2017). Cytochrome b5 reductase 3 is essential for cardiomyocyte function. Circulation, 136(suppl_1), A20733-A20733.

Rahaman, M. M., Nguyen, A. T., Miller, M. P., Hahn, S. A., Sparacino-Watkins, C., Jobbagy, S., ... & Straub, A. C. (2017). Cytochrome b5 reductase 3 modulates soluble guanylate cyclase redox state and cGMP signaling. Circulation research, 121(2), 137-148.

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Protocols

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)
Proteogenomics
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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