Anti-Human Perforin Antibody (DMAB5438MH)

Mouse anti-Human Perforin Monoclonal antibody [clone 6C11] for IHC Datasheet

Online Inquiry Add to basket

Specifications


Host Species
Mouse
Antibody Isotype
IgG1
Clone
6C11
Immunogen
Recombinant protein corresponding to an external region of the Cterminus of the perforin molecule.
Conjugate
Unconjugated

Target


Alternative Names
PRF1; perforin 1 (pore forming protein); P1; PFP; FLH2; PFN1
Entrez Gene ID
UniProt ID

Product Background


Gene summary
PRF1 (Perforin 1) is a Protein Coding gene. Diseases associated with PRF1 include hemophagocytic lymphohistiocytosis, familial, 2 and aplastic anemia. Among its related pathways are Platelet activation, signaling and aggregation and Signaling by GPCR. GO annotations related to this gene include calcium ion binding and wide pore channel activity. 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.
Antigen Description
Perforin-1 is a protein that in humans is encoded by the PRF1 gene. Familial hemophagocytic lymphohistiocytosis 2 (FHL2) [MIM:603553]: A rare disorder characterized by immune dysregulation with hypercytokinemia, defective function of natural killer cell, and massive infiltration of several organs by activated lymphocytes and macrophages. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, and less frequently neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits and ataxia. Note=The disease is caused by mutations affecting the gene represented in this entry. Perforin-1 is a protein that in humans is encoded by the PRF1 gene and the Prf1 gene in mice. Perforin is a pore forming cytolytic protein found in the granules of Cytotoxic T lymphocytes (CTLs) and NK cells. Upon degranulation, perforin binds to the target cells plasma membrane, and oligomerises in a Ca2+ dependent manner to form pores on the target cell. The pore formed allows for the passive diffusion of a family of pro-apoptotic proteases, known as the granzymes, into the target cell. The lytic membrane-inserting part of perforin is the MACPF domain. This region shares homology with cholesterol-dependent cytolysins from Gram-positive bacteria.
Pathway
Allograft rejection; Apoptosis; Autoimmune thyroid disease; Caspase cascade in apoptosis; Downstream signaling in naive CD8+ T cells; Graft-versus-host disease; IL12 signaling mediated by STAT4; IL2 signaling events mediated by.

Citations


Have you cited DMAB5438MH in a publication? Let us know and earn a reward for your research.

References


Perricone, C; Ceccarelli, F; et al. An overview on the genetic of rheumatoid arthritis: A never-ending story. AUTOIMMUNITY REVIEWS 10:599-608(2011).
Strout, MP; Seropian, S; et al. Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis. NATURE REVIEWS CLINICAL ONCOLOGY 7:415-420(2010).

Custom Antibody Labeling


We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody. Learn More

Customer Reviews


Write a review, share your experiences with others and get rewarded !

Online Inquiry

Name
Phone *
E-mail Address *
Service & Products Interested *
Project Description
Verification Code * Please input "diagnostics" as verification code.

Online Inquiry

Order Info: Anti-Human Perforin Antibody

Online Inquiry
  Interested in larger quantities ? request a quote!
  Protocol may be improved. Please feel free to contact us to obtain the latest version.!
  15% off your first purchase

Ordering Information

Payment methods we support:
Invoice / Purchase Order
Credit card

OUR PROMISE TO YOU Guaranteed product quality expert customer support

Inquiry Basket