Perforin 1 rabbit pAb

The protein encoded by this gene has structural and functional similarities to complement component 9 (C9). Like C9, this protein creates transmembrane tubules and is capable of lysing non-specifically a variety of target cells. This protein is one of the main cytolytic proteins of cytolytic granules, and it is known to be a key effector molecule for T-cell- and natural killer-cell-mediated cytolysis. Defects in this gene cause familial hemophagocytic lymphohistiocytosis type 2 (HPLH2), a rare and lethal autosomal recessive disorder of early childhood. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008],

Product Specifications

Background

The protein encoded by this gene has structural and functional similarities to complement component 9 (C9) . Like C9, this protein creates transmembrane tubules and is capable of lysing non-specifically a variety of target cells. This protein is one of the main cytolytic proteins of cytolytic granules, and it is known to be a key effector molecule for T-cell- and natural killer-cell-mediated cytolysis. Defects in this gene cause familial hemophagocytic lymphohistiocytosis type 2 (HPLH2), a rare and lethal autosomal recessive disorder of early childhood. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008]

Product Name Alternative

Perforin-1 (P1) (Cytolysin) (Lymphocyte pore-forming protein) (PFP)

UniProt

P14222

Swiss Prot

P14222

Reactivity

Human; Rat; Mouse

Immunogen

The antiserum was produced against synthesized peptide derived from the C-terminal region of human PRF1. AA range:451-500

Target

Perforin

Clonality

Polyclonal

Source

Rabbit

Applications

WB; IHC; ELISA

Concentration

1 mg/ml

Dilution

WB 1:500-2000, IHC 1:50-200, ELISA 1:10000-20000

Buffer

-20°C/1 year

Molecular Weight

61kD

Storage Conditions

-20°C/1 year

Observed Molecular Weight

61kD

Fragment

IgG

Subcellular Location

Cytolytic granule . Secreted. Cell membrane ; Multi-pass membrane protein . Endosome lumen . Stored in cytolytic granules of cytolytic T-lymphocytes and secreted into the cleft between T-lymphocyte and target cell (PubMed:20038786) . Inserts into the cell membrane of target cells and forms pores (PubMed:20889983) . Membrane insertion and pore formation requires a major conformation change (PubMed:20889983) . May be taken up via endocytosis involving clathrin-coated vesicles and accumulate in a first time in large early endosomes (PubMed:20038786) . .