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Product Name SPTBN2 Chinese Name 非红细胞血影脑蛋白β2/spectrin β III抗体 Alias Beta III spectrin; Beta-III spectrin; SCA 5; SCA5; Spectrin beta chain brain 2; Spectrin beta non erythrocytic 2; Spectrin non erythroid beta chain 2; Spinocerebellar ataxia 5; SPTBN 2; Spectrin beta chain, non-erythrocytic 2; Beta-III spectrin; Spinocerebellar ataxia 5 protein; SPTN2_HUMAN. Research Area Neurobiology Signal transduction Cytoskeleton Immunogen Species Rabbit Clonality Polyclonal React Species Human, Rat, (predicted: Mouse, Dog, Cow, Rabbit, Sheep, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 271kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human SPTBN2: 71-170/2390 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Spectrin is an actin binding protein that is a major component of the plasma membrane skeleton. Spectrins function as membrane organizers and stabilizers by forming dimers, tetramers and higher polymers. Spectrin Alpha and spectrin Beta are present in erythrocytes, whereas spectrin Alpha II (also designated fodrin Alpha) and spectrin Beta I (also designated fodrin Beta) are present in other somatic cells. The spectrin tetramers in erythrocytes act as barriers to lateral diffusion, but spectrin dimers seem to lack this function. Spectrin Beta III is highly homologous to both spectrin Beta I and spectrin Beta II. Western blot analysis shows that spectrin Beta III migrates at a higher molecular mass than predicted in the kidney. Spectrin Beta III is highly expressed in brain, kidney, pancreas, and liver, and at lower levels in lung and placenta. Specifically, spectrin Beta III constitutes a major component of the Golgi and vesicular membrane skeletons.
Function:
Probably plays an important role in neuronal membrane skeleton.
Subcellular Location:
Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.
Tissue Specificity:
Highly expressed in brain, kidney, pancreas, and liver, and at lower levels in lung and placenta.
DISEASE:
Defects in SPTBN2 are the cause of spinocerebellar ataxia type 5 (SCA5) [MIM:600224]. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA5 is an autosomal dominant cerebellar ataxia (ADCA). It is a slowly progressive disorder with variable age at onset, ranging between 10 and 50 years.
Similarity:
Belongs to the spectrin family.
Contains 2 CH (calponin-homology) domains.
Contains 1 PH domain.
Contains 17 spectrin repeats.
SWISS:
O15020
Gene ID:
6712
Database links:Entrez Gene: 6712 Human
Omim: 604985 Human
SwissProt: O15020 Human
Unigene: 26915 Human
Product Picture Tissue/cell: human kidney tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-SPTBN2 Polyclonal Antibody, Unconjugated(SL10784R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-SPTBN2 Polyclonal Antibody, Unconjugated(SL10784R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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