Description:

Size: 100ul

Catalog no.: bs-15483R-A594

Price: 380 EUR

Product details

Modification Site

None

Gene ID Number

11112

Crossreactivity

Human

Target Antigen

HIBADH

Tested applications

IF(IHC-P)

French translation

anticorps

Clonality

Polyclonal

Modification

Unmodified

Concentration

1ug per 1ul

Excitation emission

590nm/617nm

Conjugated with

ALEXA FLUOR® 594

Conjugated

Alexa conjugate 1

Clone

Polyclonal antibody

Recommended dilutions

IF(IHC-P)(1:50-200)

Purification

Purified by Protein A.

Category

Conjugated Primary Antibodies

Conjugation

Alexa Fluor,ALEXA FLUOR® 594

Host Organism

Rabbit (Oryctolagus cuniculus)

Also known as

Anti-HIBADH PAb ALEXA FLUOR 594

Specificity

This is a highly specific antibody against HIBADH.

Long name

HIBADH Polyclonal Antibody, ALEXA FLUOR 594 Conjugated

Cross-reactive species details

Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.

Source

This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human HIBADH

Storage conditions

Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.

Synonyms

3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN.

Properties

For facs or microscopy Alexa 1 conjugate.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.

Background of the antigen

HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.