Rabbit Anti-Nucleoside phosphorylase/FITC Conjugated antibody

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Inosine phosphorylase; MGC117396; MGC125915; MGC125916; NP; Np1; Nucleoside phosphorylase; PNP; Pnp1; PNPH_HUMAN; PRO1837; PUNP; Purine nucleoside orthophosphate ribosyltransferase; Purine nucleoside phosphorylase; FLJ94043; FLJ97288.

Cat:

SL11739R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human PNP/Nucleoside phosphorylase

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

32kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
Purine nucleoside phosphorylase (PNP), also designated inosine phosphorylase, forms a homotrimer. It belongs to the PNP/MTAP phosphorylase family of proteins. Human PNP catalyzes the reversible phosphorolysis of ribonucleosides and 2’-deoxyribonucleosides with specificity for guanine, hypoxanthine, and their analogs. PNP deficiency is a rare autosomal recessive genetic disease associated with a severe defect in T-lymphocyte function and neurologic disorder in children, comprising four percent of combined immunodeficiency cases. Children with PNP deficiency are highly prone to infections, autoimmune disorders, neurological impairment, and cancer.

Function:
Defects in PNP are the cause of purine nucleoside phosphorylase deficiency (PNP deficiency) [MIM:613179]. It leads to a severe T-cell immunodeficiency with neurologic disorder in children.

Subunit:
Homotrimer.

Subcellular Location:
Cytoplasm, cytoskeleton (By similarity).Cytoplasm

Tissue Specificity:
Expressed in red blood cells; overexpressed inred blood cells (cytoplasm) of patients with hereditarynon-spherocytic hemolytic anemia of unknown etiology.

DISEASE:
Defects in PNP are the cause of purine nucleosidephosphorylase deficiency (PNPD) [MIM:613179]. It leads to a severeT-cell immunodeficiency with neurologic disorder in children.

Similarity:
Belongs to the PNP/MTAP phosphorylase family.

Database links:

Entrez Gene: 4860 Human

Omim: 112850 Human

SwissProt: P00491 Human

Unigene: 75514 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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