Recombinant Human XPNPEP3 protein (ab173067)
- Datasheet
- References
- Protocols
Overview
-
Product nameRecombinant Human XPNPEP3 protein
See all XPNPEP3 proteins and peptides -
Protein lengthFull length protein
Description
-
NatureRecombinant
-
SourceEscherichia coli
-
Amino Acid Sequence
-
Accession
-
SpeciesHuman
-
SequenceMGSSHHHHHHSSGLVPRGSHMPWLLSAPKLVPAVANVRGLSGCMLCSQRR YSLQPVPERRIPNRYLGQPSPFTHPHLLRPGEVTPGLSQVEYALRRHKLM SLIQKEAQGQSGTDQTVVVLSNPTYYMSNDIPYTFHQDNNFLYLCGFQEP DSILVLQSLPGKQLPSHKAILFVPRRDPSRELWDGPRSGTDGAIALTGVD EAYTLEEFQHLLPKMKAETNMVWYDWMRPSHAQLHSDYMQPLTEAKAKSK NKVRGVQQLIQRLRLIKSPAEIERMQIAGKLTSQAFIETMFTSKAPVEEA FLYAKFEFECRARGADILAYPPVVAGGNRSNTLHYVKNNQLIKDGEMVLL DGGCESSCYVSDITRTWPVNGRFTAPQAELYEAVLEIQRDCLALCFPGTS LENIYSMMLTLIGQKLKDLGIMKNIKENNAFKAARKYCPHHVGHYLGMDV HDTPDMPRSLPLQPGMVITIEPGIYIPEDDKDAPEKFRGLGVRIEDDVVV TQDSPFILSADCPKEMNDIEQICSQASLEHHHHHH
-
Molecular weight60 kDa including tags
-
Amino acids1 to 507
-
TagsHis tag C-Terminus , His tag N-Terminus
-
Associated products
-
Related Products
Specifications
Our Abpromise guarantee covers the use of ab173067 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
-
Applications
HPLC
SDS-PAGE
-
Endotoxin level< 1.000 Eu/µg
-
Purity>95% by SDS-PAGE .
ab173067 is greater than 95% pure, as determined by SEC-HPLC and reducing SDS-PAGE. -
FormLiquid
-
Concentration information loading...
Preparation and Storage
-
Stability and Storage
Shipped on Dry Ice. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.30
Constituents: 0.02% DTT, 0.3% Tris
It is supplied as an 0.2 µM filtered solution.
General Info
-
Alternative names
- Aminopeptidase P3
- APP3
- NPHPL1
see all -
Tissue specificityIsoform 1 and isoform 2 are widely expressed, with isoform 1 being more abundant.
-
Involvement in diseaseDefects in XPNPEP3 are the cause of nephronophthisis-like nephropathy type 1 (NPHPL1) [MIM:613159]. A 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, mental retardation and hypertrophic dilated cardiomyopathy.
-
Sequence similaritiesBelongs to the peptidase M24B family.
-
Cellular localizationMitochondrion.
- Information by UniProt
Datasheets and documents
References
ab173067 has not yet been referenced specifically in any publications.
