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Product Name FGGY Chinese Name 肌萎缩侧索硬化症相关蛋白FGGY抗体 Alias fggy; FGGY carbohydrate kinase domain containing; FGGY carbohydrate kinase domain-containing protein; FGGY_HUMAN; FLJ10986; MGC94804; OTTHUMP00000010078; OTTHUMP00000010081; OTTHUMP00000010082; OTTHUMP00000202071; RP11-242B9.1. Research Area Cell biology Neurobiology Signal transduction Alzheimer's Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, Rat, (predicted: Human, Dog, Cow, Horse, Rabbit, Sheep, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 60kDa Cellular localization The nucleus cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human FGGY: 151-250/551 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail FGGY is a 551 amino acid member of the FGGY kinase family that exists as four isoforms which are produced by alternative splicing events. Expressed in lung, kidney, small intestine, liver and fetal brain, FGGY is encoded by a gene that maps to chromosome 1 and, when mutated, is associated with sporadic amyotrophic lateral sclerosis (ALS). ALS is a neurodegenerative disorder that affects motor neurons and results in fatal paralysis, usually within 2 to 5 years after initial diagnosis. Chromosome 1, on which the gene encoding FGGY is located, is the largest human chromosome, spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, many of which are associated with genetic diseases, including Hutchinson-Gilford progeria, familial adenomatous polyposis, Stickler syndrome, Gaucher disease and Usher syndrome.
Function:
Expressed in kidney, lung and small intestine and to a lower extent in liver and detected in cerebrospinal fluid (at protein level).
Tissue Specificity:
Expressed in fetal brain (at protein level).
DISEASE:
Defects in FGGY are associated with sporadic amyotrophic lateral sclerosis (ALS) [MIM:105400]. Amyotrophic lateral sclerosis is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors.
Similarity:
Belongs to the FGGY kinase family.
SWISS:
Q96C11
Gene ID:
55277
Database links:Entrez Gene: 772383 Chicken
Entrez Gene: 100067351 Horse
Entrez Gene: 55277 Human
Entrez Gene: 75578 Mouse
Entrez Gene: 406567 Zebrafish
Omim: 611370 Human
SwissProt: Q96C11 Human
SwissProt: A2AJL3 Mouse
Product Picture Paraformaldehyde-fixed, paraffin embedded (mouse liver tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (FGGY) Polyclonal Antibody, Unconjugated (SL11689R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.Paraformaldehyde-fixed, paraffin embedded (rat kidney tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (FGGY) Polyclonal Antibody, Unconjugated (SL11689R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.Paraformaldehyde-fixed, paraffin embedded (rat tongue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (FGGY) Polyclonal Antibody, Unconjugated (SL11689R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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