CSTB antibody - N-terminal region (ARP45749_P050)

Product Specifications

CAS Number

9007-83-4

Gene Name

Cystatin B (stefin B)

Gene Aliases

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

Gene ID

1476

Swiss Prot

P04080

Accession Number

NP_000091

Reactivity

Human, Mouse, Rat, Cow, Guinea Pig, Horse, Pig, Rabbit, Sheep

Immunogen

The immunogen is a synthetic peptide directed towards the N terminal region of human CSTB

Target

CSTB is 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 CSTB gene are responsible for the primary defects in patients with progressive myoclonic epilepsy 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.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) .

Partner Proteins

UBC; SARNP; DPP7; BAG3; CFTR; ECT2; SEC24A; NUDC; MAT2A; FLNB; DCUN1D1; CUL3; RAD21; SPRY2; CTSL; CTSD; CTSH; CTSB; CST3; VHL

Clonality

Polyclonal

Type

Polyclonal Antibody

Sequence

MMCGAPSATQPATAETQHIADQVRSQLEEKENKKFPVFKAVSFKSQVVAG

Applications

IHC, WB

Purification

Affinity Purified

Assay Protocol

Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol