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FGGY Rabbit pAb (bs-11689R)  
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產品編號 bs-11689R
英文名稱 FGGY Rabbit pAb
中文名稱 肌萎縮側索硬化癥相關蛋白FGGY抗體
別    名 fggy; FGGY carbohydrate kinase domain containing; FGGY carbohydrate kinase domain-containing protein; FGGY_HUMAN; FLJ10986; MGC94804; OTTHUMP00000010078; OTTHUMP00000010081; OTTHUMP00000010082; OTTHUMP00000202071; RP11-242B9.1.  
研究領域 細胞生物  神經生物學  信號轉導  Alzheimer's  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,Rat (predicted: Human,Rabbit,Sheep,Cow,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 60 kDa
檢測分子量
細胞定位 細胞核 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FGGY: 151-250/551 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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: 534011 Cow

Entrez Gene: 100067351 Horse

Entrez Gene: 55277 Human

Entrez Gene: 75578 Mouse

Entrez Gene: 298250 Rat

Entrez Gene: 406567 Zebrafish

Omim: 611370 Human

SwissProt: Q96C11 Human

SwissProt: A2AJL3 Mouse



產品圖片
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 (bs-11689R) 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 (bs-11689R) 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 (bs-11689R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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