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Aldehyde dehydrogenase 18 family, member A1

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, Jul 2008] (from NCBI)
Top mentioned proteins: CAN, ACID, V1a, HAD, Insulin
Papers on GPR
Gestational protein restriction alters cell proliferation in rat placenta.
Catisti et al., Araras, Brazil. In J Mol Histol, Feb 2016
UNASSIGNED: We recently showed that gestational protein restriction (GPR) alters the structure of the rat placenta on day 19 of gestation (dG).
Sulphide Resistance in the Cyanobacterium Microcystis aeruginosa: a Comparative Study of Morphology and Photosynthetic Performance Between the Sulphide-Resistant Mutant and the Wild-Type Strain.
Flores-Moya et al., Málaga, Spain. In Microb Ecol, Jan 2016
The irradiance-saturated photosynthetic oxygen-production rate (GPR max) and the photosynthetic efficiency (measured both by oxygen production and fluorescence, α (GPR) and α (ETR)) were lower in the S (r) strain than in the wild-type.
Development of glycine-α-methyl-proline-containing tripeptides with neuroprotective properties.
Patruno et al., Chieti, Italy. In Eur J Med Chem, Jan 2016
The syntheses of GP(Me)R, GP(Me)K, and GP(Me)H were all achieved both by introducing the Pro(Me) residue into the Gly-Pro-Arg (GPR) sequence in place of the native Pro in P2 position and replacing the basic amino acid Arg in P3 position by Lys or His.
[The trend of new drug development for the treatment of diabetes mellitus].
Kaku, In Nihon Rinsho, Dec 2015
The hypoglycemic drugs such as glucagon receptor antagonists, glucokinase activators, and GPR agonists and oral medication of GLP-1 receptor agonist are under phase 2 trial in Japan.
The Intestinal Microbiota Contributes to the Ability of Helminths to Modulate Allergic Inflammation.
Harris et al., Lausanne, Switzerland. In Immunity, Dec 2015
The helminth-induced anti-inflammatory cytokine secretion and regulatory T cell suppressor activity that mediated the protection required the G protein-coupled receptor (GPR)-41.
Tamoxifen resistance: from cell culture experiments towards novel biomarkers.
Kalinski et al., Magdeburg, Germany. In Pathol Res Pract, Mar 2015
Tamoxifen resistance is associated with altered estrogen receptor expression especially on the plasma membrane, including the alternative G-protein coupled receptor GPR-30 (GPER) and estrogen receptor splice products, such as ERα36.
The role of kisspeptin signalling in the hypothalamic-pituitary-gonadal axis - current perspective.
Sathyapalan et al., Kingston upon Hull, United Kingdom. In Endokrynol Pol, 2014
We will also be appraising other factors that regulate the kiSS1/Kisspeptin/GPR-54 system.
[Niacin deficiency and cutaneous immunity].
Sugita et al., Switzerland. In Nihon Rinsho Meneki Gakkai Kaishi, 2014
Niacin binds with G protein-coupled receptor (GPR) 109A on cutaneous Langerhans cells and causes vasodilation with flushing in head and neck area.
Mitochondria-controlled signaling mechanisms of brain protection in hypoxia.
Kirova et al., Moscow, Russia. In Front Neurosci, 2014
Patterns of mitochondria-controlled activation of GPR-91- and HIF-1-dependent reaction were considered, and a possibility of their participation in cellular-intercellular-systemic interactions in hypoxia and adaptation was proved.
Polarity establishment, asymmetric division and segregation of fate determinants in early C. elegans embryos.
Gönczy et al., Davis, United States. In Wormbook, 2013
We explain how a ternary complex comprised of Gα (GOA-1/GPA-16), GPR-1/GPR-2, and LIN-5 is essential for anchoring the motor protein dynein to the cell cortex, where it is thought to exert pulling forces on depolymerizing astral microtubules.
G protein signaling modulator-3: a leukocyte regulator of inflammation in health and disease.
Tarrant et al., Chapel Hill, United States. In Am J Clin Exp Immunol, 2013
G protein signaling modulator-3 (GPSM3), also known as G18 or AGS4, is a member of a family of proteins containing one or more copies of a small regulatory motif known as the GoLoco (or GPR) motif.
aPKC phosphorylates NuMA-related LIN-5 to position the mitotic spindle during asymmetric division.
van den Heuvel et al., Utrecht, Netherlands. In Nat Cell Biol, 2011
In animals, an evolutionarily conserved pathway of LIN-5 (homologues: Mud and NuMA), GPR-1/2 (homologues: Pins, LGN, AGS-3) and Gα mediates spindle positioning, and acts downstream of the conserved PAR-3-PAR-6-aPKC polarity complex.
Further expansion of the phenotypic spectrum associated with mutations in ALDH18A1, encoding Δ¹-pyrroline-5-carboxylate synthase (P5CS).
Robertson et al., Halifax, Canada. In Am J Med Genet A, 2011
expansion of the phenotypic spectrum associated with mutations in ALDH18A1
Codon adaptation-based control of protein expression in C. elegans.
Bringmann et al., Dresden, Germany. In Nat Methods, 2011
We found that the force acting on the spindle in C. elegans embryos was related to the amount of the G-protein regulator GPR-1/2.
Association of variants within APOE, SORL1, RUNX1, BACE1 and ALDH18A1 with dementia in Alzheimer's disease in subjects with Down syndrome.
Prasher et al., Coventry, United Kingdom. In Neurosci Lett, 2011
ALDH18A1 genetic variants are associated with Down syndrome in subjects with dementia of Alzheimer's disease.
NuMA-related LIN-5, ASPM-1, calmodulin and dynein promote meiotic spindle rotation independently of cortical LIN-5/GPR/Galpha.
van den Heuvel et al., Utrecht, Netherlands. In Nat Cell Biol, 2009
Spindle positioning is controlled by an evolutionarily conserved pathway, which involves LIN-5/GPR-1/2/Galpha in Caenorhabditis elegans, Mud/Pins/Galpha in Drosophila and NuMA/LGN/Galpha in humans.
Human Delta1-pyrroline-5-carboxylate synthase: function and regulation.
Valle et al., Albuquerque, United States. In Amino Acids, 2008
analysis of function and regulation of Delta1-pyrroline-5-carboxylate synthase
A missense mutation in ALDH18A1, encoding Delta1-pyrroline-5-carboxylate synthase (P5CS), causes an autosomal recessive neurocutaneous syndrome.
Robertson et al., Dunedin, New Zealand. In Eur J Hum Genet, 2008
These data suggest that P5CS may possess additional uncharacterised functions that affect connective tissue and central nervous system function.
Coupling of cortical dynein and G alpha proteins mediates spindle positioning in Caenorhabditis elegans.
Gönczy et al., Lausanne, Switzerland. In Nat Cell Biol, 2007
We found that the G alpha-associated proteins GPR-1/2 and LIN-5 interact in vivo with LIS-1, a component of the dynein complex.
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