ALDH7A1 Antibody (PACO56720)
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주문- SKU:
- PACO56720
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Rat
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Application:
- IP
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
Antibody Name: | ALDH7A1 Antibody (PACO56720) |
Antibody SKU: | PACO56720 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC, IF, IP |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:200-1:500, IF:1:50-1:200, IP:1:200-1:2000, |
Species Reactivity: | Human, Rat, Mouse |
Immunogen: | Recombinant Human Alpha-aminoadipic semialdehyde dehydrogenase protein (386-515AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | Western Blot. Positive WB detected in: Hela whole cell lysate, HepG2 whole cell lysate, 293T whole cell lysate, SH-SY5Y whole cell lysate, Rat liver tissue, Rat kidney tissue, Mouse liver tissue, Mouse kidney tissue. All lanes: ALDH7A1 antibody at 4.7µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 59, 56, 52 kDa. Observed band size: 59 kDa. |
![]() | IHC image of PACO56720 diluted at 1:400 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
![]() | Immunofluorescence staining of Hela cells with PACO56720 at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
Background: | Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism. |
Synonyms: | Alpha-aminoadipic semialdehyde dehydrogenase (Alpha-AASA dehydrogenase) (EC 1.2.1.31) (Aldehyde dehydrogenase family 7 member A1) (EC 1.2.1.3) (Antiquitin-1) (Betaine aldehyde dehydrogenase) (EC 1.2.1.8) (Delta1-piperideine-6-carboxylate dehydrogenase) (P6c dehydrogenase), ALDH7A1, ATQ1 |
UniProt Protein Function: | ALDH7A1: Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism. Defects in ALDH7A1 are the cause of pyridoxine-dependent epilepsy (PDE). PDE is characterized by a combination of various seizure types. It usually occurs in the first hours of life and is unresponsive to standard anticonvulsants, responding only to immediate administration of pyridoxine hydrochloride. Belongs to the aldehyde dehydrogenase family. 3 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - histidine; Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - ascorbate and aldarate; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - propanoate; Carbohydrate Metabolism - pyruvate; EC 1.2.1.3; EC 1.2.1.31; EC 1.2.1.8; Lipid Metabolism - fatty acid; Lipid Metabolism - glycerolipid; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Oxidoreductase; Secondary Metabolites Metabolism - limonene and pinene degradation Chromosomal Location of Human Ortholog: 5q31 Cellular Component: cytoplasm; mitochondrial matrix; mitochondrion Molecular Function:aldehyde dehydrogenase (NAD) activity; betaine-aldehyde dehydrogenase activity; L-aminoadipate-semialdehyde dehydrogenase activity; protein binding Biological Process: aldehyde metabolic process; choline catabolic process; lysine catabolic process; sensory perception of sound Disease: Epilepsy, Pyridoxine-dependent |
NCBI Summary: | The protein encoded by this gene is a member of subfamily 7 in the aldehyde dehydrogenase gene family. These enzymes are thought to play a major role in the detoxification of aldehydes generated by alcohol metabolism and lipid peroxidation. This particular member has homology to a previously described protein from the green garden pea, the 26g pea turgor protein. It is also involved in lysine catabolism that is known to occur in the mitochondrial matrix. Recent reports show that this protein is found both in the cytosol and the mitochondria, and the two forms likely arise from the use of alternative translation initiation sites. An additional variant encoding a different isoform has also been found for this gene. Mutations in this gene are associated with pyridoxine-dependent epilepsy. Several related pseudogenes have also been identified. [provided by RefSeq, Jan 2011] |
UniProt Code: | P49419 |
NCBI GenInfo Identifier: | 982742419 |
NCBI Gene ID: | 501 |
NCBI Accession: | |
UniProt Secondary Accession: | P49419,O14619, Q6IPU8, Q9BUL4, B2R669, B4DIC7, B4DMA0 E7EPT3, |
UniProt Related Accession: | P49419 |
Molecular Weight: | 51,411 Da |
NCBI Full Name: | alpha-aminoadipic semialdehyde dehydrogenase isoform 3 |
NCBI Synonym Full Names: | aldehyde dehydrogenase 7 family member A1 |
NCBI Official Symbol: | ALDH7A1 |
NCBI Official Synonym Symbols: | EPD; PDE; ATQ1 |
NCBI Protein Information: | alpha-aminoadipic semialdehyde dehydrogenase |
UniProt Protein Name: | Alpha-aminoadipic semialdehyde dehydrogenase |
UniProt Synonym Protein Names: | Aldehyde dehydrogenase family 7 member A1 (EC:1.2.1.3); Antiquitin-1; Betaine aldehyde dehydrogenase (EC:1.2.1.8); Delta1-piperideine-6-carboxylate dehydrogenase; P6c dehydrogenase |
Protein Family: | Alpha-aminoadipic semialdehyde dehydrogenase |
UniProt Gene Name: | ALDH7A1 |
UniProt Entry Name: | AL7A1_HUMAN |