This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene. [provided by RefSeq]||Applications: |Suitable for use in Western Blot. Other applications not tested.||Recommended Dilution:|Optimal dilutions to be determined by the researcher.||AA Sequence:|MLSQVYRCGFQPFNQHLLPWVKCTTVFRSHCIQPSVI RHVRSWSNIPFITVPLSRTHGKSFAHRSELKHAKRIVV KLGSAVVTRGDECGLALGRLASIVEQVSVLQNQGREM MLVTSGAVAFGKQRLRHEILLSQSVRQALHSGQNQLK EMAIPVLEARACAAAGQSGLMALYEAMFTQYSICAAQI LVTNLDFHDEQKRRNLNGTLHELLRMNIVPIVNTNDAV VPPAEPNSDLQGVNVISVKDNDSLAARLAVEMKTDLLI VLSDVEGLFDSPPGSDDAKLIDIFYPGDQQSVTFGTKS RVGMGGMEAKVKAALWALQGGTSVVIANGTHPKVSG HVITDIVEGKKVGTFFSEVKPAGPTVEQQGEMARSGG RMLATLEPEQRAEIIHHLADLLTDQRDEILLANKKDLEE AEGRLAAPLLKRLSLSTSKLNSLAIGLRQIAASSQDSVG RVLRRTRIAKNLELEQVTVPIGVLLVIFESRPDCLPQVA ALAIASGNGLLLKGGKEAAHSNRILHLLTQEALSIHGVK EAVQLVNTREEVEDLCRLDKMIDLIIPRGSSQLVRDIQK AAKGIPVMGHSEGICHMYVDSEASVDKVTRLVRDSKC EYPAACNALETLLIHRDLLRTPLFDQIIDMLRVEQVKIHA GPKFASYLTFSPSEVKSLRTEYGDLELCIEVVDNVQDA IDHIHKYGSSHTDVIVTEDENTAEFFLQHVDSACVFWN ASTRFSDGYRFGLGAEVGISTSRIHARGPVGLEGLLTT KWLLRGKDHVVSDFSEHGSLKYLHENLPIPQRNTN||Storage and Stability:|May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.Product Type:Pab|Isotype: IgG|Host: mouse|Source: human|Concentration: As reported |Form: Supplied as a liquid in PBS, pH 7.4.|Purity: Purified|Immunogen: Protein corresponding to aa1-795 from full length human ALDH18A1|Specificity: Recognizes human ALDH18A1||Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.

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