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Mouse Anti-SUOX Recombinant Antibody (CBXS-1026) (CBMAB-S3919-CQ)

This product is a mouse antibody that recognizes SUOX. The antibody CBXS-1026 can be used for immunoassay techniques such as: S-ELISA, WB, RNAi, ELISA, WB.
See all SUOX antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-1026
Antibody Isotype
IgG1, κ
Application
S-ELISA, WB, RNAi, ELISA, WB

Basic Information

Immunogen
SUOX (AAH65193, 1 a.a.-488 a.a) Full length recombinant protein with GST tag
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 7.2
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
SUOX
Introduction
Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins.
Entrez Gene ID
6821
UniProt ID
P51687
Alternative Names
Sulfite Oxidase
Function
Catalyzes the oxidation of sulfite to sulfate, the terminal reaction in the oxidative degradation of sulfur-containing amino acids.
Biological Process
Sulfur compound metabolic process
Cellular Location
Mitochondrion intermembrane space
Involvement in disease
Sulfite oxidase deficiency, isolated (ISOD):
A life-threatening, autosomal recessive neurometabolic disorder characterized by severe neurological impairment. Classic ISOD manifests in the first few hours to days of life and is characterized by intractable seizures, feeding difficulties, rapidly progressive encephalopathy, microcephaly, and profound intellectual disability. Children usually die during the first few months of life. Mild ISOD manifests in infancy or early childhood and is characterized by ectopia lentis that is variably present, developmental delay and regression, movement disorder characterized by dystonia and choreoathetosis, ataxia, and rarely acute hemiplegia due to metabolic stroke.
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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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