Name: U-Blot® Perforin Rabbit mAb
SKU: WR5059
Price: 268 USD
Availability: InStock

U-Blot® Perforin Rabbit mAb

Ucallm_WR5059-50

Price range: $268.00 through $328.00

Size

100μl

50μl

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SKU: WR5059-100

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Product details

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],

Specifications

Size	50μL/100μL
Target	Perforin
Reactivity	Mouse, Rat
Applications	WB, IF, IP, ELISA
MW(Calculated)	62kD
MW(Observed)	62kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200;
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM1294R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	PRF1
Protein Name	Perforin-1
Other Name	Cytolysin; / FLH2; / HPLH2; / Lymphocyte pore-forming protein; / P1; / PERF_HUMAN; / perforin 1; / pore forming protein; / Perforin 1; / Perforin-1; / PFP; / PGFL; / PIGF; / PIGF-2; / PLGF; / Pore forming protein; / prf1; / SHGC-10760
Species	Human
Gene ID	5551
Uniprot	P14222,
Species.1	Mouse
Gene ID.1	18646
Uniprot.1	P10820,
Species.2	Rat
Gene ID.2
Uniprot.2	P35763
Cellular Localization	Cytoplasmic, Membranous
Tissue Expression	Spleen
Signaling_pathway	Cellular Processes >> Cell growth and death >> Apoptosis;Organismal Systems >> Immune system >> Natural killer cell mediated cytotoxicity;Human Diseases >> Immune disease >> Autoimmune thyroid disease;Human Diseases >> Immune disease >> Allograft rejection;Human Diseases >> Immune disease >> Graft-versus-host disease
Research Areas	>>Apoptosis; / >>Natural killer cell mediated cytotoxicity; / >>Type I diabetes mellitus; / >>Autoimmune thyroid disease; / >>Allograft rejection; / >>Graft-versus-host disease; / >>Viral myocarditis
Function	Disease:Defects in PRF1 are the cause of familial hemophagocytic lymphohistiocytosis type 2 (FHL2) [MIM:603553]; also known as HPLH2. Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous, rare autosomal recessive disorder. It is characterized by immune dysregulation with hypercytokinemia and defective natural killer cell function. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits, and ataxia. Hemophagocytosis is a prominent feature of the disease, and a non-malignant infiltration of macrophages and activated T lymphocytes in lymph nodes, spleen, and other organs is also found.,Function:In the presence of calcium, perforin polymerizes into transmembrane tubules and is capable of lysing non-specifically a variety of target cells.,induction:Repressed by contact with target cells.,online information:Perforin entry,online information:PRF1 mutation db,similarity:Belongs to the complement C6/C7/C8/C9 family.,similarity:Contains 1 C2 domain.,similarity:Contains 1 EGF-like domain.,similarity:Contains 1 MACPF domain.,subcellular location:Cytoplasmic granules of cytolytic T-lymphocytes.,