GLUD1 Antibody

說明書

中文名稱：

GLUD1兔多克隆抗體
貨號：

CSB-PA009544GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

P00367
基因名：

GLUD1
別名：

AI118167 antibody; DHE3_HUMAN antibody; EC 1.4.1.3 antibody; GDH 1 antibody; GDH antibody; Gdh-X antibody; GDH1 antibody; GLUD antibody; Glud1 antibody; Glud1a antibody; Glud1b antibody; Gludl antibody; Glutamate dehydrogenase (NAD(P)+) antibody; Glutamate dehydrogenase 1 antibody; Glutamate dehydrogenase 1 mitochondrial antibody; Glutamate dehydrogenase 1a antibody; Glutamate dehydrogenase 1b antibody; Memory-related gene 2 protein antibody; MGC127177 antibody; MGC132003 antibody; MGC80801 antibody; MGC93608 antibody; mitochondrial antibody; MRG-2 antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human GLUD1
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Mitochondrial glutamate dehydrogenase that catalyzes the conversion of L-glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. Plays a role in insulin homeostasis. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate.
基因功能參考文獻：
1. This study provides insight into the molecular mechanism by which GDH1-mediated metabolic reprogramming of glutaminolysis mediates lung cancer metastasis and offers a therapeutic strategy for patients with LKB1-deficient lung cancer. PMID: 29249655
2. Phenotypic heterogeneity of GDH-hyperinsulinemia patients was observed within the Chinese cohort in the present study. The fact that most patients had a GLUD1 p. S445L mutation implies that this site could be a hotspot in Chinese patients. PMID: 30306091
3. structural model of a complex of human Sirt4 and GDH in order to elucidate the molecular mechanism underlying the ADP-ribosylation of GDH by Sirt4 PMID: 29571013
4. Upregulation of GLUD1 and SLC25A13 was associated with tumor aggressiveness and poorer prognosis in colorectal cancer patients. PMID: 27924922
5. This study showed that SIRT5 supports the anaplerotic entry of glutamine into the TCA cycle in malignant phenotypes of CRC via activating GLUD1. PMID: 29416026
6. the present study shows that the GDHS445L enzyme is highly sensitive to the allosteric activator ADP, explaining the hyperactivity of this mutant. INS-1E beta-cells expressing GDHS445L responded to glutamine stimulation by increasing the mitochondrial respiration and ATP production, as well as by a rise in cytosolic calcium. PMID: 28911206
7. We have shown that alpha-ketoglutarate substrate inhibition kinetics of GDH, which include both random and obligatory ordered association/dissociation reactions, robustly control the ratio between glutamate and ammonium under a wide range of intracellular substrate variation. Dysregulation of this activity under pericentral nitrogen insufficiency contributes to the breaking down of ammonia homeostasis and thereby can s... PMID: 27747338
8. mRNA and protein levels of GluD1 were increased in iPSC-derived neurons from FOXG1(+/-) patients. PMID: 26443267
9. Hypoxia-induced expression of GDH relies on the up-regulation of HIF1alpha but not HIF2alpha. HIF1alpha binds the promoter of GDH and promotes the transcription of GDH gene in lung cancer cells. PMID: 28065856
10. Results indicate that it is possible to use high-throughput screening methods to find activators for glutamate dehydrogenase (GDH) that might be useful as pharmaceutical agents. PMID: 27808506
11. Missense mutation of GLUD1 is associated with Hyperinsulinism-hyperammonemia syndrome. PMID: 27383869
12. Analysis of Serbian patients with Hyperinsulinism/hyperammonemia syndrome confirms the association of p.S445L and p.R221C mutations with hypoglycaemic seizures noted within the first three months of life and with subsequent risk for cognitive impairment and/or epilepsy. PMID: 26759084
13. GLUD1 is differentially expressed in the cellular and subcellular compartments of numerous tissues. PMID: 27422263
14. GLUD1 mutation is associated with congenital hyperinsulinism-hyperammonemia. PMID: 26656609
15. Study of the expression of the GDH1/2 in human steroidogenic organs revealed that, while GDH2 was expressed specifically in steroid-synthesizing cells, GDH1 was expressed both in the cells that produce steroids and in those that lack endocrine function. PMID: 26241911
16. GDH plays a critical role in colorectal cancer progression PMID: 25947346
17. Mutation in the GLUD1 gene is associated with hyperinsulinism/hyperammonemia. PMID: 25781533
18. side-chain interactions between 409 and 443 positions in the 'antenna' region of GDH are crucial for basal catalytic activity, allosteric regulation, and relative resistance to thermal inactivation PMID: 25620628
19. argeting GDH1 by shRNA or a small molecule inhibitor R162 resulted in imbalanced redox homeostasis, leading to attenuated cancer cell proliferation and tumor growth. PMID: 25670081
20. SIRT3 and glutamate dehydrogenase are altered in follicular cells of women with reduced ovarian reserve or advanced maternal age PMID: 24771001
21. The GDH1 is a key metabolic enzyme with emerging roles in insulin regulation. MitoNEET forms a covalent complex with GDH1 through disulfide bond formation and acts as an activator. PMID: 24295216
22. The data suggest a dual mechanism by which glutamate dehydrogenase activity modulates autophagy, i.e., by activating MTORC1 and by limiting the formation of reactive oxygen species. PMID: 23575388
23. first N-terminal alpha helical structure is crucial for the mitochondrial import of hGDH2 and these findings may have implications in understanding the evolutionary mechanisms that led to the large mitochondrial targeting signals of human GDHs PMID: 22658952
24. Lack of Glud1 in transgenic mouse brain inhibits GDH activity regardless of enzymatic direction, modified glutamate handling, decreased glutamate catabolism and increased glutamine levels without affecting synaptic transmission. PMID: 22924626
25. Transgenic mice are useful in defining the molecular pathways affected by the over-activation of GLUD transgene in glutamatergic neurons of the brain and spinal cord. PMID: 21397652
26. Human GDH1 appears to act like bovine GDH1, but human GDH2 does not show the same enhancement of branched chain alpha-keto acid dehydrogenase complex enzyme activities. PMID: 21621574
27. [review] Hyperinsulinemic hypoglycemia and hyperammonemia demonstrate the important role of GDH in insulin regulation and illustrate unexpectedly important reasons for the unusually complex allosteric regulation of GDH. PMID: 21130127
28. GDH interactions with aristolochic acid induce apoptosis of renal tubular epithelial cells. PMID: 21901531
29. Report glutamate dehydrogenase mutations in Japanese patients with congenital hyperinsulinism and hyperammonemia syndrome. PMID: 22106762
30. Green tea polyphenols control dysregulated glutamate dehydrogenase in transgenic mice by hijacking the ADP activation site. PMID: 21813650
31. In Chinese, R269H, S445L mutation of the GLUD1 leads to congenital hyperinsulinism. PMID: 20931523
32. Gradual decrease in leukocyte GLDH activity may be a key factor for neurodegenerative aging processes. PMID: 20197649
33. Two missense mutations give rise to two different forms of congenital hyperinsulinism/hyperammonemia. PMID: 11840195
34. increased glutamate dehydrogenase activity confers on beta-cells the ability to secrete insulin in response to glutamine PMID: 11872671
35. Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations PMID: 11903050
36. identify the structural basis for allosteric differences of GlUD1 and GLUD2 PMID: 11950837
37. Tyr187 is responsible for the efficient base binding of ADP to human GDH. PMID: 12022886
38. site-directed mutagenesis showing involvement of Glu(279)in NAD+ binding PMID: 12193607
39. substitution of Ser for Arg-443 (but not substitution of Thr for Ser-331, Leu for Met-370, or Leu for Met-415) virtually abolished basal activity and totally abrogated the activation of the enzyme by l-leucine (1-10 mm) in the absence of other effectors PMID: 12324473
40. These results suggest that the Ser443 residue plays an important role in the different thermal stability of human glutamate dehydrogenase isozymes (hGDH1 and hGDH2). PMID: 15044002
41. inhibition of glutamate dehydrogenase results in sulfite neurotoxicity PMID: 15273247
42. GLUD2 originated by retroposition from GLUD1 in the hominoid ancestor less than 23 million years ago. PMID: 15378063
43. results suggest that cysteine 323 plays an important role in catalysis by human GDH isozymes; C323 is not directly involved in allosteric regulation. PMID: 15750346
44. GLUD1 may have a role in hyperinsulinism/hyperammonaemia syndrome PMID: 16151898
45. Results describe the electrophysiological mechanisms underlying the dysregulated insulin secretion in pancreatic beta cells with glutamate dehydrogenase mutations. PMID: 16492972
46. we describe a family with a dominantly inherited mutation in GDH in Myoclonic absence epilepsy with photosensitivity. PMID: 18321734
47. GLUD1 parental protein localizes to mitochondria and the cytoplasm, GLUD2 is specifically targeted to mitochondria. PMID: 18688271
48. Although GDH activity is also highly expressed in the brain, central nervous system (CNS) manifestations of GLUD1 activating mutations have remained relatively unexplored. PMID: 19046183
49. Neurological disorders in HHS are more frequent than previously thought and might suggest that mutations in the GTP binding site of GDH could be associated with more frequent epilepsy. PMID: 19046187
50. Hence, while most of the hGDHs translocate into the mitochondria (a process associated with cleavage of the signal sequence), part of the protein localizes to the endoplasmic reticulum, probably serving additional functions PMID: 19448744
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相關疾病：

Familial hyperinsulinemic hypoglycemia 6 (HHF6)
亞細胞定位：

Mitochondrion. Endoplasmic reticulum.
蛋白家族：

Glu/Leu/Phe/Val dehydrogenases family
數據庫鏈接：

HGNC: 4335

OMIM: 138130

KEGG: hsa:2746

STRING: 9606.ENSP00000277865

UniGene: Hs.500409

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