Name: Anti-CSTB Antibody (CAB19961)[KO Validated]
Brand: KO Validated Antibodies 2
SKU: CAB19961
Availability: OutOfStock

Antibody Name:	Anti-CSTB Antibody [KO Validated]
Antibody SKU:	CAB19961
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Rat
Host Species:	Rabbit
Immunogen:	Recombinant protein of human CSTB.

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Rat
Positive Samples:	293T

Immunogen:	Recombinant protein of human CSTB.
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	Email for sequence
Gene ID:	1476
Uniprot:	P04080
Cellular Location:	Cytoplasm, Nucleus
Calculated MW:	11kDa
Observed MW:	13KDa

Anti-CSTB Antibody [KO Validated] (CAB19961)

Western blot analysis of extracts from normal (control) and CSTB knockout (KO) 293T cells, using CSTB antibody (CAB19961) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.

Synonyms:

CSTB, CPI-B, CST6, EPM1, EPM1A, PME, STFB, ULD

Background:

The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG repeat from 2-3 copies to 30-78 copies.

UniProt Protein Function:	CSTB: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1). EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Belongs to the cystatin family.
UniProt Protein Details:	Protein type:Inhibitor Chromosomal Location of Human Ortholog: 21q22.3 Cellular Component: extracellular space; cytoplasm; nucleolus Molecular Function:protease binding; endopeptidase inhibitor activity; cysteine protease inhibitor activity Biological Process: negative regulation of proteolysis; regulation of apoptosis; adult locomotory behavior; negative regulation of peptidase activity Disease: Myoclonic Epilepsy Of Unverricht And Lundborg
NCBI Summary:	The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). [provided by RefSeq, Jul 2008]
UniProt Code:	P04080
NCBI GenInfo Identifier:	1706278
NCBI Gene ID:	1476
NCBI Accession:	P04080.2
UniProt Related Accession:	P04080
Molecular Weight:	11,140 Da
NCBI Full Name:	Cystatin-B
NCBI Synonym Full Names:	cystatin B (stefin B)
NCBI Official Symbol:	CSTB
NCBI Official Synonym Symbols:	PME; ULD; CST6; EPM1; STFB; EPM1A
NCBI Protein Information:	cystatin-B; CPI-B; liver thiol proteinase inhibitor
UniProt Protein Name:	Cystatin-B
UniProt Synonym Protein Names:	CPI-B; Liver thiol proteinase inhibitor; Stefin-B
Protein Family:	Cystatin
UniProt Gene Name:	CSTB
UniProt Entry Name:	CYTB_HUMAN

Antibodies	ELISA Kits
Anti-CSTB Antibody (CAB3815)	Human Cystatin-B (CSTB) ELISA Kit
CSTB Antibody (PACO00675)	Stefin B Colorimetric Cell-Based ELISA Kit
CSTB Antibody (PACO08647)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)