Sku: WR8148
U-Blot® Perforin Rabbit mAb
Catalog No.: WR5059
Specification: 50μL/100μL
Stock: In stock
U-Blot® Perforin Rabbit mAb Learn more
Product information
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 Overview
- Catalog No.
- WR5059
- SKU
- WR5059-50
- Category
- Rabbit mAbs
- Product Type
- Other
- Size
- 50μL/100μL
- Stock Status
- In stock
- Available Stock
- 20
- Minimum Order Quantity
- 1
Storage & Compliance
- Storage
- -15°C to -25°C/1 year(Do not lower than -25°C)
- Research Use Only
- Yes
Additional Specifications
- Target
- Perforin
- Reactivity
- Mouse,Rat
- Application
- WB,IF,IP,ELISA
- MW(Calculated)
- 62kD
- MW(Observed)
- 62kD
- Host Species
- Rabbit
- Isotype
- IgG,Kappa
- Conjugate/Modification
- Unmodified
- Modification site
- --
- Recommended Dilution Ratio
- WB 1:2000-1:10000;IF 1:200-1:1000;ELISA 1:5000-1:20000;IP 1:50-1:200;
- Form
- PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
- Source
- --
- Purification
- Protein A
- Purity
- --
- storageCondition
- -15°C to -25°C/1 year(Do not lower than -25°C)
- Concentration
- --
- Clonality
- Monoclonal
- Clone No.
- PT1294R
- Immunogen
- --
- Sequence
- --
- 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
- Organism-1
- Human
- Gene ID-1
- 5551
- SwissProt-1
- P14222
- Organism-2
- Mouse
- Gene ID-2
- 18646
- SwissProt-2
- P10820
- Organism-3
- Rat
- Gene ID-3
- --
- SwissProt-3
- P35763
- Organism-4
- --
- gene ID-4
- --
- SwissProt-4
- --
- Cellular Localization
- 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.,
Tech Support
ucallm
Documents
Datasheet, COA, SDS, and protocol files can be requested from technical support.
Storage & Handling
- Storage
- -15°C to -25°C/1 year(Do not lower than -25°C)
Contact Support
Emailinfo@ucallmlabs.com
Phone+(1)-866-986-9598
WeChat / IMUcallm-Tech
HoursMonday-Friday 09:00-18:00 CST
