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Mouse Anti-XPNPEP3 Recombinant Antibody (2D5) (CBMAB-X0162-YC)

Provided herein is a Mouse monoclonal antibody against Human XPNPEP3. The antibody, clone 2D5, can be used for immunoassay techniques, such as WB, IHC, IF, FC.
See all XPNPEP3 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Dog, Rat, Monkey, Mouse
Clone
2D5
Antibody Isotype
IgG2b
Application
WB, IHC, IF, FC

Basic Information

Immunogen
Full length recombinant protein of human XPNPEP3(NP_071381) produced in HEK293 cell
Specificity
Human, Dog, Rat, Monkey, Mouse
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
X-prolyl aminopeptidase 3
Introduction
XPNPEP3 belongs to the family of X-pro-aminopeptidases that utilize a metal cofactor, and remove the N-terminal amino acid from peptides with a proline residue in the penultimate position. XPNPEP3 localizes to the mitochondria of renal cells, and have a role in ciliary function. Mutations in XPNPEP3 are associated with nephronophthisis-like nephropathy-1.
Entrez Gene ID
Human63929
Mouse321003
Rat685823
Dog481237
Monkey705778
UniProt ID
HumanQ9NQH7
MouseB7ZMP1
RatB5DEQ3
DogA0A8I3PGA7
MonkeyH9F7L1
Alternative Names
APP3; ICP55; NPHPL1
Function
Catalyzes the removal of a penultimate prolyl residue from the N-termini of peptides, such as Leu-Pro-Ala (PubMed:25609706, PubMed:28476889).
Also shows low activity towards peptides with Ala or Ser at the P1 position (PubMed:28476889).
Isoform 1
Promotes TNFRSF1B-mediated phosphorylation of MAPK8/JNK1 and MAPK9/JNK2, suggesting a function as an adapter protein for TNFRSF1B; the effect is independent of XPNPEP3 peptidase activity. May inhibit apoptotic cell death induced via TNF-TNFRSF1B signaling.
Biological Process
Biological Process glomerular filtration Source:MGI1 Publication
Biological Process protein processing Source:MGI1 Publication
Biological Process proteolysis Source:UniProtKB1 Publication
Cellular Location
Isoform 1
Mitochondrion
Cytoplasm
Mainly mitochondrial. Translocates to the cytoplasm following TNFRSF1B activation.
Isoform 2
Cytoplasm
Involvement in disease
Nephronophthisis-like nephropathy 1 (NPHPL1):
An autosomal recessive disorder with features of nephronophthisis, a cystic kidney disease leading to end-stage renal failure. Nephronophthisis is histologically characterized by modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts. Typical clinical manifestation are chronic renal failure, anemia, polyuria, polydipsia, isosthenuria, and growth retardation. Associations with extrarenal symptoms are frequent. In NPHPL1 patients, extrarenal symptoms include hypertension, essential tremor, sensorineural hearing loss and gout. Severely affected individuals can manifest a mitochondrial disorder with isolated complex I deficiency activity in muscle, seizures, intellectual disability and hypertrophic dilated cardiomyopathy.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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