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Rabbit Anti-ALDH5A1 Recombinant Antibody (BA0340) (CBMAB-0059CQ)


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Published Data
Rabbit Anti-ALDH5A1 Recombinant Antibody (BA0340) in WB
Biochemical characterization of the Leu243Pro variant. (A) Semi-quantitative polymerase chain reaction (PCR) analysis of ALDH5A1 gene expression of two independent control subjects and the patient harboring the c.278G> T and c.728T> C variants. (B) Western blot analysis of succinic semialdehyde dehydrogenase (SSADH) protein expression in dermal fibroblasts. (C) SSADH-deficient HEK-293T cells (SSADH-KO-HEK-293T) were transiently transfected with either wildtype (WT) SSADH or p.Leu243Pro constructs. After 48 h, cell lysates were prepared and subjected to Western blotting (C) and SSADH enzyme activity assay (D). (C) Representative Western blot analysis of SSADH protein levels. Glyceraldehyde 3-phosphate dehydrogenase (GAPDH) was used as a loading control. n = 3. (D) SSADH enzyme activity was assessed fluorometrically. WT activity was set to 100%. Data are expressed as mean ± SD of three independent experiments. Student's t test: *** p ≤ 0.01. ...View More
Brennenstuhl, H., Didiasova, M., Assmann, B., Bertoldi, M., Molla, G., Jung-Klawitter, S., ... & Tikkanen, R. (2020). Succinic Semialdehyde Dehydrogenase Deficiency: In Vitro and In Silico Characterization of a Novel Pathogenic Missense Variant and Analysis of the Mutational Spectrum of ALDH5A1. International journal of molecular sciences, 21(22), 8578.
Rabbit Anti-ALDH5A1 Recombinant Antibody (BA0340) in Immunoblot
Interneuron hTau overexpression downregulated total GAT1 and d-GABAAR but exhibited limited effects on many other proteins involved in either GABA synthesis, transport, release, degradation, and post-synaptic signaling. ...View More
Zheng, J., Li, H. L., Tian, N., Liu, F., Wang, L., Yin, Y., ... & Wang, J. Z. (2020). Interneuron accumulation of phosphorylated tau impairs adult hippocampal neurogenesis by suppressing GABAergic transmission. Cell Stem Cell, 26(3), 331-345.
Rabbit Anti-ALDH5A1 Recombinant Antibody (BA0340) in IHC
GABA oxidation and SSADH expression differences in glioma cell lines; and common SSADH expression in human glioblastoma/glioma samples. a Oxygen consumption rates after using GABA - as an energy substrate - in U251 wt, U251 IDH1m, U87 MG and U373 MG cells in D5030 (representative oxygen consumption curves - Seahorse measurements). b SSADH expression in U251 wt, U251 IDH1m, U87 MG and U373 MG cells (representative Western blot results). c Summarised data about the evaluation of SSADH expressions in wt and IDH1m human samples (upper diagram) and in different human glioma types (lower diagram) and representative IHC stainings with HE counterstaining (DAB-brown staining, magnification 200×; normal human brain tissue and kidney were used as controls). ...View More
Hujber, Z., Horváth, G., Petővári, G., Krencz, I., Dankó, T., Mészáros, K., ... & Sebestyén, A. (2018). GABA, glutamine, glutamate oxidation and succinic semialdehyde dehydrogenase expression in human gliomas. Journal of Experimental & Clinical Cancer Research, 37, 1-12.

Summary

Host Animal
Rabbit
Specificity
Human, Mouse
Clone
BA0340
Antibody Isotype
IgG
Application
WB, IHC-P

Basic Information

Immunogen
Synthetic peptide within Human ALDH5A1/SSADH aa 500-600.
Host Species
Rabbit
Specificity
Human, Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:10,000-1:50,000
IHC-P1:100-1:250

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Supernatant
Buffer
9% PBS, 40% Glycerol, 0.05% BSA
Preservative
0.01% sodium azide
Concentration
Batch dependent
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Aldehyde Dehydrogenase 5 Family Member A1
Introduction
This protein belongs to the aldehyde dehydrogenase family of proteins. The ALDH5A1 gene contains 10 exons. 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 a consanguineous family in which 2 sibs and their cousin had SSADH deficiency (271980). The mutation resulted in deletion of exon 9 and a frameshift after amino acid 401 followed by 52 nonsense residues before a stop codon was reached. The 2 sibs in this family exhibited hypotonia in addition to developmental and speech delays, hyporeflexia, and behavioral problems, including mild autism.
Entrez Gene ID
Human7915
Mouse214579
Rat291133
UniProt ID
HumanP51649
MouseQ8BWF0
RatP51650
Alternative Names
Aldehyde Dehydrogenase 5 Family, Member A1; NAD(+)-Dependent Succinic Semialdehyde Dehydrogenase; EC 1.2.1.24; SSADH; Mitochondrial Succinate Semialdehyde Dehydrogenase; Aldehyde Dehydrogenase 5 Family Member A1; Aldehyde Dehydrogenase Family 5 Member A1; Succinate-Semialdehyde Dehydrogenase; EC 1.2.1; SSDH
Function
Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).
Biological Process
Central nervous system development Source: UniProtKB
Gamma-aminobutyric acid catabolic process Source: UniProtKB
Glutamate metabolic process Source: UniProtKB
Neurotransmitter catabolic process Source: UniProtKB
Post-embryonic development Source: Ensembl
Succinate metabolic process Source: UniProtKB
Cellular Location
Mitochondrion
Involvement in disease
Succinic semialdehyde dehydrogenase deficiency (SSADHD): A rare inborn error of 4-aminobutyric acid (GABA) metabolism, which leads to accumulation of 4-hydroxybutyric acid in physiologic fluids of patients. The disease is clinically characterized by developmental delay, hypotonia, mental retardation, ataxia, seizures, hyperkinetic behavior, aggression, and sleep disturbances.
More Infomation

Yoganathan, S., Arunachal, G., Kratz, L., Varman, M., Thomas, M., Sudhakar, S. V., ... & Danda, S. (2021). Metabolic or ischemic stroke in succinic semi-aldehyde dehydrogenase deficiency due to the homozygous variant c. 1343+ 1_1343+ 3delGTAinsTT in ALDH5A1. Annals of Indian Academy of Neurology, 24(2), 259.

Fattahi, M., Bushehri, A., Alavi, A., Asghariazar, V., Nozari, A., Firouzabadi, S. G., ... & Behjati, F. (2020). Bi-allelic Mutations in ALDH5A1 is associated with succinic semialdehyde dehydrogenase deficiency and severe intellectual disability. Gene, 144918.

Vernau, K. M., Struys, E., Letko, A., Woolard, K. D., Aguilar, M., Brown, E. A., ... & Bannasch, D. L. (2020). A Missense Variant in ALDH5A1 Associated with Canine Succinic Semialdehyde Dehydrogenase Deficiency (SSADHD) in the Saluki Dog. Genes, 11(9), 1033.

Cao, C., Wang, X., Li, R., Jin, P., Chen, H., Xia, M., ... & Tian, X. (2020). Downregulation of ALDH5A1 Promotes Tumor Metastasis and Contributes to Poor Prognosis in Ovarian Cancer.

Menduti, G., Biamino, E., Vittorini, R., Vesco, S., Puccinelli, M. P., Porta, F., ... & Rossi, L. (2018). Succinic semialdehyde dehydrogenase deficiency: The combination of a novel ALDH5A1 gene mutation and a missense SNP strongly affects SSADH enzyme activity and stability. Molecular genetics and metabolism, 124(3), 210-215.

Tian, X., Han, Y., Yu, L., Luo, B., Hu, Z., Li, X., ... & Ma, D. (2017). Decreased expression of ALDH5A1 predicts prognosis in patients with ovarian cancer. Cancer biology & therapy, 18(4), 245-251.

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For research use only. Not intended for any clinical use.

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