Anti-PAH antibody (ab106805)
Key features and details
- Goat polyclonal to PAH
- Suitable for: WB
- Reacts with: Rat, Human
- Isotype: IgG
Overview
-
Product name
Anti-PAH antibody
See all PAH primary antibodies -
Description
Goat polyclonal to PAH -
Host species
Goat -
Tested applications
Suitable for: WBmore details -
Species reactivity
Reacts with: Rat, Human
Predicted to work with: Mouse, Chicken, Cow, Dog -
Immunogen
Synthetic peptide:
ESRPSRLKKDE
by a Cysteine residue linker, corresponding to internal sequence amino acids 66-76 of Human PAH (NP_000268.1). -
Positive control
- WB: Human liver and rat kidney tissue lysates.
-
General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
-
Form
Liquid -
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles. -
Storage buffer
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA -
Concentration information loading...
-
Purity
Immunogen affinity purified -
Purification notes
ab106805 is purified from Goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide. -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
-
Compatible Secondaries
-
Isotype control
-
Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab106805 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
---|---|---|
WB |
Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 48 kDa (predicted molecular weight: 52 kDa).
1 hour primary incubation is recommended for this product. |
Notes |
---|
WB
Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 48 kDa (predicted molecular weight: 52 kDa). 1 hour primary incubation is recommended for this product. |
Target
-
Pathway
Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6. -
Involvement in disease
Defects in PAH are the cause of phenylketonuria (PKU) [MIM:261600]. PKU is an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol (normal concentration 100 mumol) which usually causes mental retardation (unless low phenylalanine diet is introduced early in life). They tend to have light pigmentation, rashes similar to eczema, epilepsy, extreme hyperactivity, psychotic states and an unpleasant 'mousy' odor.
Defects in PAH are the cause of non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA) [MIM:261600]. Non-PKU HPA is a mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Non-PKU HPA is usually caused by the combined effect of a mild hyperphenylalaninemia mutation and a severe one.
Defects in PAH are the cause of hyperphenylalaninemia (HPA) [MIM:261600]. HPA is the mildest form of phenylalanine hydroxylase deficiency. -
Sequence similarities
Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
Contains 1 ACT domain. - Information by UniProt
-
Database links
- Entrez Gene: 408024 Chicken
- Entrez Gene: 510583 Cow
- Entrez Gene: 475446 Dog
- Entrez Gene: 5053 Human
- Entrez Gene: 18478 Mouse
- Entrez Gene: 24616 Rat
- Omim: 612349 Human
- SwissProt: Q2KIH7 Cow
see all -
Alternative names
- PAH antibody
- PH antibody
- PH4H_HUMAN antibody
see all
Images
-
Anti-PAH antibody (ab106805) at 0.1 µg/ml + Human liver tissue lysate (35µg protein in RIPA buffer)
Developed using the ECL technique.
Predicted band size: 52 kDa -
Anti-PAH antibody (ab106805) at 0.3 µg/ml + Rat kidney tissue lysate (35µg protein in RIPA buffer)
Developed using the ECL technique.
Predicted band size: 52 kDa
Datasheets and documents
-
SDS download
-
Datasheet download
References (0)
ab106805 has not yet been referenced specifically in any publications.