Aldehyde Dehydrogenase 5 Family Member A1 (ALDH5A1) Antibody

292.5€ (80 µl)
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935106861
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name
Aldehyde Dehydrogenase 5 Family Member A1 (ALDH5A1) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx028256
tested applications
ELISA, WB, IHC
Description
ALDH5A1 belongs to the aldehyde dehydrogenase family of proteins. This protein functions as a mitochondrial NAD (+) dependent succinic semialdehyde dehydrogenase. A deficiency of this enzyme, known as 4-hydroxybutyricaciduria, is a rare inborn error in the metabolism of the neurotransmitter 4-aminobutyric acid (GABA). In response to the defect, physiologic fluids from patients accumulate GHB, a compound with numerous neuromodulatory properties.
Documents del producto
Instrucciones
Data sheet
Product specifications
Category | Primary Antibodies |
Immunogen Target | Aldehyde Dehydrogenase 5 Family Member A1 (ALDH5A1) |
Host | Rabbit |
Reactivity | Human |
Recommended Dilution | WB: 1/1000, IHC-P: 1/10 - 1/50. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
Clonality | Polyclonal |
Conjugation | Unconjugated |
Isotype | IgG |
Purification | Purified through a protein A column, followed by peptide affinity purification. |
Size 1 | 80 µl |
Size 2 | 400 µl |
Form | Liquid |
Tested Applications | ELISA, WB, IHC |
Buffer | PBS containing 0.09% sodium azide. |
Availability | Shipped within 5-10 working days. |
Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
Dry Ice | No |
UniProt ID | P51649 |
Alias | ALDH5A1,SSADH |
Background | Antibody anti-ALDH5A1 |
Status | RUO |
Descripción
ALDH5A1, also known as succinic semialdehyde dehydrogenase (SSADH), is a mitochondrial enzyme involved in the catabolism of gamma-aminobutyric acid (GABA), an important inhibitory neurotransmitter. ALDH5A1 catalyzes the oxidation of succinic semialdehyde (SSA) to succinate, a key intermediate in the tricarboxylic acid (TCA) cycle, linking GABA metabolism to cellular energy production. Mutations in ALDH5A1 result in succinic semialdehyde dehydrogenase deficiency, a rare autosomal recessive disorder that causes the accumulation of SSA and GABA, leading to neurological symptoms such as developmental delays, hypotonia, seizures, and ataxia. ALDH5A1 plays a crucial role in maintaining GABA homeostasis and preventing neurotoxicity. Its dysfunction highlights the importance of proper neurotransmitter balance and energy metabolism in neurological health. Therapies targeting ALDH5A1 function focus on reducing SSA accumulation and alleviating associated neurological impairments.
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