U-Blot® Perforin Rabbit mAb

U-Blot® Perforin Rabbit mAb
U-Blot® Perforin Rabbit mAb
$268.00
Size:50μL
SKU: WR5059-50

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

01

Documents

Datasheet, COA, SDS, and protocol files can be requested from technical support.

02

Storage & Handling

Storage
-15°C to -25°C/1 year(Do not lower than -25°C)
06

Contact Support

Emailinfo@ucallmlabs.com

Phone+(1)-866-986-9598

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Keywords:Rabbit mAbs