Aldehyde Dehydrogenase 5 Family Member A1 (ALDH5A1) Antibody

Este producto es parte de ALDH - Aldehyde dehydrogenase family member
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292.5€ (80 µl)

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935106861
info@markelab.com
name
Aldehyde Dehydrogenase 5 Family Member A1 (ALDH5A1) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx028257
tested applications
ELISA, WB, IHC, FCM

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.

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Product specifications

CategoryPrimary Antibodies
Immunogen TargetAldehyde Dehydrogenase 5 Family Member A1 (ALDH5A1)
HostRabbit
ReactivityHuman
Recommended DilutionWB: 1/1000, IHC-P: 1/50 - 1/100, FCM: 1/10 - 1/50. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user.
ClonalityPolyclonal
ConjugationUnconjugated
IsotypeIgG
PurificationPurified through a protein A column, followed by peptide affinity purification.
Size 180 µl
Size 2400 µl
FormLiquid
Tested ApplicationsELISA, WB, IHC, FCM
BufferPBS containing 0.09% sodium azide.
AvailabilityShipped within 5-10 working days.
StorageAliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Dry IceNo
UniProt IDP51649
AliasALDH5A1,SSADH
BackgroundAntibody anti-ALDH5A1
StatusRUO

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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