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Product Details
PA1-23482
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Rabbit Polyclonal to Q(399) D K I F Q G N K D Y H K D V R N N(416) of the human ESDN Protein
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Affinity chromatography
Storage buffer
PBS
Contains
0.02% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
AB_2277084
Product Specific Information
PA1-23482 detects ESDN in Human samples.
Target Information
This protein is a key enzyme in the catabolic pathway of odd-chain fatty acids, isoleucine, threonine, methionine, and valine. It is probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. The protein is located in the mitochondrial matrix. Defects in PCCA are the cause o fpropionic acidemia type I (PA-1) [MIM:606054]. PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, develop-mental retardation, and intolerance to protein.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: 1700055P21Rik; coagulation factor V/VIII-homology domains protein 1; CUB, LCCL and coagulation factor V/VIII-homology domains protein 1; discoidin; Discoidin, CUB and LCCL domain-containing protein 2; Endothelial and smooth muscle cell-derived neuropilin-like protein
Gene Aliases: CLCP1; DCBLD2; ESDN
UniProt ID: (Human) Q96PD2
Entrez Gene ID: (Human) 131566
Performance Guarantee
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