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Mouse Anti-SDHB Recombinant Antibody (CBT2002) (V2LY-0625-LY1403)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBT2002
Antibody Isotype
IgG1
Application
IHC, FC

Basic Information

Immunogen
Purified recombinant fragment of human SDHB (AA: 29-280) expressed in E. Coli.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:500-1:2,000
IHC-P1:200-1:1,000
FC1:200-1:400
ELISA1:10,000

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

Format
Liquid
Buffer
PBS
Preservative
Sodium azide
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.

Target

Full Name
succinate dehydrogenase complex iron sulfur subunit B
Entrez Gene ID
6390
Function
Iron-sulfur protein (IP) subunit of the succinate dehydrogenase complex (mitochondrial respiratory chain complex II), responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Biological Process
Biological Process aerobic respirationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process mitochondrial electron transport, succinate to ubiquinone1 PublicationIC:ComplexPortal
Biological Process proton motive force-driven mitochondrial ATP synthesis1 PublicationIC:ComplexPortal
Biological Process respiratory electron transport chainManual Assertion Based On ExperimentIBA:GO_Central
Biological Process succinate metabolic processIEA:Ensembl
Biological Process tricarboxylic acid cycleManual Assertion Based On ExperimentTAS:ProtInc
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Pheochromocytoma (PCC):
A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent.
Paragangliomas 4 (PGL4):
A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Most commonly, they are located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.
Paraganglioma and gastric stromal sarcoma (PGGSS):
Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.
Mitochondrial complex II deficiency, nuclear type 4 (MC2DN4):
A form of mitochondrial complex II deficiency, a disorder with heterogeneous clinical manifestations. Some patients have multisystem involvement of the brain, heart, muscle, liver, and kidneys resulting in death in infancy, whereas others have only isolated cardiac or muscle involvement with onset in adulthood and normal cognition. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. MC2DN4 is a severe, autosomal recessive form characterized by early-onset progressive neurodegeneration with leukoencephalopathy.
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For research use only. Not intended for any clinical use.

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