Description
| Antibody Name: | Anti-ALDH4A1 Antibody |
| Antibody SKU: | CAB2595 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IF |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 354-563 of human ALDH4A1 (NP_003739.2). |
| Application: | WB IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse |
| Positive Samples: | HepG2, K-562, A-549, BT-474, Mouse skeletal muscle, Mouse intestine, Mouse kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 354-563 of human ALDH4A1 (NP_003739.2). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | LYVP HSLW PQIK GRLL EEHS RIKV GDPA EDFG TFFS AVID AKSF ARIK KWLE HARS SPSL TILA GGKC DDSV GYFV EPCI VESK DPQE PIMK EEIF GPVL SVYV YPDD KYKE TLQL VDST TSYG LTGA VFSQ DKDV VQEA TKVL RNAA GNFY INDK STGS IVGQ QPFG GARA SGTN DKPG GPHY ILRW TSPQ VIKE THKP LGDW SYAY MQ |
| Gene ID: | 8659 |
| Uniprot: | P30038 |
| Cellular Location: | Mitochondrion matrix |
| Calculated MW: | 55kDa/56kDa/61kDa |
| Observed MW: | 65kDa |
| Synonyms: | ALDH4A1, ALDH4, P5CD, P5CDh |
| Background: | This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. |
| UniProt Protein Function: | ALDH4A1: Irreversible conversion of delta-1-pyrroline-5- carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes. Defects in ALDH4A1 are the cause of hyperprolinemia type 2 (HP-2). HP-2 is characterized by the accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. The disorder may be causally related to neurologic manifestations, including seizures and mental retardation. Belongs to the aldehyde dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Amino Acid Metabolism - arginine and proline; Mitochondrial; EC 1.2.1.88; Amino Acid Metabolism - alanine, aspartate and glutamate; Oxidoreductase Chromosomal Location of Human Ortholog: 1p36 Cellular Component: mitochondrial matrix Molecular Function:identical protein binding; aldehyde dehydrogenase (NAD) activity; electron carrier activity; 1-pyrroline-5-carboxylate dehydrogenase activity Biological Process: proline catabolic process; 4-hydroxyproline catabolic process; proline biosynthetic process; glutamate biosynthetic process; proline catabolic process to glutamate; proline metabolic process Disease: Hyperprolinemia, Type Ii |
| NCBI Summary: | This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jun 2009] |
| UniProt Code: | P30038 |
| NCBI GenInfo Identifier: | 62511241 |
| NCBI Gene ID: | 8659 |
| NCBI Accession: | P30038.3 |
| UniProt Secondary Accession: | P30038,Q8CHT0, Q7SY23, A7YWE4, |
| UniProt Related Accession: | P30038 |
| Molecular Weight: | 61719 |
| NCBI Full Name: | Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial |
| NCBI Synonym Full Names: | aldehyde dehydrogenase 4 family member A1 |
| NCBI Official Symbol: | ALDH4A1Â Â |
| NCBI Official Synonym Symbols: | P5CD; ALDH4; P5CDh  |
| NCBI Protein Information: | delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial |
| UniProt Protein Name: | Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial |
| UniProt Synonym Protein Names: | Aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenase |
| UniProt Gene Name: | ALDH4A1Â Â |
| UniProt Entry Name: | AL4A1_HUMAN |
 | Western blot analysis of extracts of various cell lines, using ALDH4A1 antibody (CAB2595) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. |
 | Immunofluorescence analysis of A549 cells using ALDH4A1 antibody (CAB2595). |
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