ALDH5A1
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Official Full Name
aldehyde dehydrogenase 5 family, member A1
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Overview
This protein belongs to the aldehyde dehydrogenase family of proteins. This gene encodes 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. Two transcript variants encoding distinct isoforms have been identified for this gene. -
Synonyms
ALDH5A1; aldehyde dehydrogenase 5 family, member A1; succinate-semialdehyde dehydrogenase, mitochondrial; SSADH; SSDH; succinate semialdehyde dehydrogenase; aldehyde dehydrogenase family 5 member A1; mitochondrial succinate semialdehyde dehydrogenase; NAD;
- Recombinant Proteins
- Cell & Tissue Lysates
- Human
- Zebrafish
- E.coli
- In Vitro Cell Free System
- Mammalian Cell
- Wheat Germ
- Yeast
- GST
- His
- SUMO
- N/A
Species | Cat.# | Product name | Source (Host) | Tag | Protein Length | Price |
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Human | ALDH5A1-2821H | Recombinant Human ALDH5A1, His-tagged | E.coli | His | ||
Human | ALDH5A1-450H | Recombinant Human ALDH5A1 Protein, GST-tagged | Wheat Germ | GST | ||
Human | ALDH5A1-27041TH | Recombinant Human ALDH5A1, His-tagged | E.coli | His | 488 amino acids | |
Human | ALDH5A1-9562H | Recombinant Human ALDH5A1, His-tagged | E.coli | His | 175-535a.a. | |
Human | ALDH5A1-26496TH | Recombinant Human ALDH5A1, His-tagged | E.coli | His | ||
Human | ALDH5A1-60HCL | Recombinant Human ALDH5A1 cell lysate | N/A | |||
Human | ALDH5A1-2508H | Recombinant Human ALDH5A1 protein, His-SUMO-tagged | E.coli | His-SUMO | 48-535aa | |
Human | ALDH5A1-1324H | Recombinant Human ALDH5A1 Protein (48-535 aa), His-tagged | Yeast | His | 48-535 aa | |
Human | ALDH5A1-1412HF | Recombinant Full Length Human ALDH5A1 Protein, GST-tagged | In Vitro Cell Free System | GST | 535 amino acids | |
Zebrafish | ALDH5A1-6188Z | Recombinant Zebrafish ALDH5A1 | Mammalian Cell | His |
- Involved Pathway
- Protein Function
- Interacting Protein
- ALDH5A1 Related Articles
ALDH5A1 involved in several pathways and played different roles in them. We selected most pathways ALDH5A1 participated on our site, such as Alanine, aspartate and glutamate metabolism, Butanoate metabolism, Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with ALDH5A1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
Pathway Name | Pathway Related Protein |
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Alanine, aspartate and glutamate metabolism | GPT;GLULC;GLSA;RIMKLB;ASPA;ASL;GFPT1;GLUL;GAD1B |
Butanoate metabolism | OXCT2;BDH2;OXCT2B;HADHAA;ACAT2;HADH;AACS;BDH1;ACSM3 |
Metabolic pathways | ALDOB;POC1B-GALNT4;POLR1B;PLCB1;POC1BL;NADKA;SEPHS1;POLR3F;PLA2G2F |
ALDH5A1 has several biochemical functions, for example, aldehyde dehydrogenase (NAD) activity, protein homodimerization activity, succinate-semialdehyde dehydrogenase (NAD+) activity. Some of the functions are cooperated with other proteins, some of the functions could acted by ALDH5A1 itself. We selected most functions ALDH5A1 had, and list some proteins which have the same functions with ALDH5A1. You can find most of the proteins on our site.
Function | Related Protein |
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aldehyde dehydrogenase (NAD) activity | ALDH3A2A;ALDH9A1B;ALDH1L1;ALDH9A1;ALDH3A2;ALDH7A1;ALDH16A1;ALDH1A1;ALDH2 |
protein homodimerization activity | TERFA;QDPR;LMLN;MTMR2;SNX2;DMRTA2;STOM;BCL2L1;SYNE1 |
succinate-semialdehyde dehydrogenase (NAD+) activity | |
succinate-semialdehyde dehydrogenase [NAD(P)+] activity |
ALDH5A1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with ALDH5A1 here. Most of them are supplied by our site. Hope this information will be useful for your research of ALDH5A1.
NR3C1
- Q&As
- Reviews
Q&As (13)
Ask a questionAlong with its primary role in GABA metabolism, ALDH5A1 may participate in other metabolic pathways. It has been suggested to be involved in the detoxification of certain aldehydes, although the extent of its contribution to these pathways is not fully understood. Further research is needed to elucidate the involvement of ALDH5A1 in other metabolic processes.
Deficiency or mutations in ALDH5A1 result in succinic semialdehyde dehydrogenase deficiency (SSADH), also known as GABA-transaminase deficiency. This condition leads to the accumulation of elevated levels of SSA and GABA-related metabolites, impacting GABAergic neurotransmission. SSADH is characterized by various neurological symptoms, including developmental delays, intellectual disability, seizures, ataxia, hypotonia, and behavioral abnormalities.
ALDH5A1 is involved in the GABA metabolic pathway, as it plays a crucial role in converting succinic semialdehyde to succinate. It is also implicated in oxidative stress defense mechanisms and has been linked to neurodevelopmental processes, such as neuronal migration and axon guidance.
ALDH5A1 plays a crucial role in the metabolism of gamma-aminobutyric acid (GABA), a neurotransmitter in the central nervous system. It catalyzes the conversion of succinic semialdehyde (SSA) to succinate, preventing the accumulation of SSA and GABA-related metabolites. Thus, ALDH5A1 acts as an important enzyme in the GABA degradation pathway, maintaining proper GABA levels and neuronal function.
Yes, there is ongoing research into the development of small molecules and inhibitors that can modulate ALDH5A1 activity for therapeutic purposes. Targeting ALDH5A1 may offer potential treatments for neurological disorders, cancer, and other conditions related to ALDH5A1 dysregulation.
Yes, mutations in the ALDH5A1 gene are associated with succinic semialdehyde dehydrogenase deficiency (SSADH). These mutations can lead to reduced enzyme activity or protein instability, resulting in the accumulation of toxic levels of succinic semialdehyde and GABA-related metabolites.
ALDH5A1 activity and expression levels could serve as biomarkers to predict drug response and optimize treatment strategies. Determining an individual's ALDH5A1 status may help personalize medication regimens for better therapeutic outcomes.
Yes, mutations or dysregulation in the ALDH5A1 gene can lead to a disorder called succinic semialdehyde dehydrogenase deficiency (SSADH). This condition disrupts GABA metabolism and is associated with various neurological symptoms, such as developmental delay, intellectual disability, and seizures.
Yes, while ALDH5A1 is primarily expressed in the brain, it is also found in other tissues, albeit at lower levels. ALDH5A1 expression has been reported in the liver, kidney, heart, and other organs, indicating its involvement in other metabolic processes outside the central nervous system.
The expression and activity of ALDH5A1 can be regulated at various levels. Transcriptional regulation through specific transcription factors can control the gene expression of ALDH5A1. Additionally, post-translational modifications, such as phosphorylation or acetylation, can modulate the protein's activity. Various signaling pathways and genetic factors can influence ALDH5A1 regulation.
ALDH5A1 deficiency is typically diagnosed through biochemical tests, such as measuring elevated levels of GABA and SSA in body fluids, including urine and cerebrospinal fluid. Genetic testing is also performed to confirm the presence of mutations in the ALDH5A1 gene. Additionally, clinical evaluation and neurological assessment may be conducted to evaluate the symptoms and presentation of the individual.
While natural inhibitors or activators specific to ALDH5A1 have not been extensively studied, certain compounds that modulate the activity or expression of aldehyde dehydrogenase enzymes in general may indirectly affect ALDH5A1 as well. For instance, some chemicals or drugs can inhibit or activate aldehyde dehydrogenases, which potentially includes ALDH5A1, depending on their specificity.
Targeting ALDH5A1 holds promise for therapeutic intervention, especially in the context of succinic semialdehyde dehydrogenase deficiency (SSADH). Developing drugs or treatments that can modulate ALDH5A1 activity or stabilize the mutated protein could potentially ameliorate the symptoms associated with this disorder. However, further research is needed to fully understand the implications and feasibility of targeting ALDH5A1 for therapeutic purposes.
Customer Reviews (4)
Write a reviewTheir expertise and assistance have been invaluable in troubleshooting and providing insightful solutions, greatly enhancing the progress of my experiments.
The ALDH5A1 Protein's superior stability and functional performance render it an indispensable tool for a wide range of scientific investigations.
They have proven to be a timely and reliable resource, readily addressing any concerns or challenges that arise during my research.
What further sets the ALDH5A1 Protein apart is the outstanding technical support provided by the manufacturer.
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