Anti-G6PC3 antibody
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概述
- 产品描述Glucose-6-phosphatase 3, also known as glucose-6-phosphatase beta, is an enzyme that in humans is encoded by the G6PC3 gene. This gene encodes the catalytic subunit of glucose 6-phosphatase (G6Pase). G6Pase is located in the endoplasmic reticulum (ER) and catalyzes the hydrolysis of glucose 6-phosphate to glucose and phosphate in the last step of the gluconeogenic and glycogenolytic pathways. G6PC3 deficiency results in a phenotypic continuum. At one end the affected individuals have only neutropenia and related complications but no other organ is affected. This is sometimes referred to as non-syndromic or isolated severe congenital neutropenia. Most affected individuals have a classic form of the disease with severe congenital neutropenia and cardiovascular and/or urogenital abnormalities. Some individuals have severe G6PC3 deficiency (also known as Dursun syndrome) and they have all the features of classic G6PC3 deficiency but in addition show involvement of non-myeloid hematopoietic cell lines, some other extra-hematologic features and pulmonary hypertension.
- 产品名称Anti-G6PC3 antibody
- 分子量39 KDa
- 种属反应性Human,Mouse,Rat,Cow,Rabbit,Sheep
- 验证应用WB
- 抗体类型兔多抗
- 免疫原KLH conjugated synthetic peptide derived from human G6PC3 201-300/346
- 偶联Non-conjugated
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性能
- 形态Liquid
- 浓度1 mg/mL.
- 存放说明Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
- 存储缓冲液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 亚型IgG
- 纯化方式Affinity purified by Protein A
- 亚细胞定位Endoplasmic reticulum membrane; Multi-pass membrane protein.
- 其它名称G-6-Pase 3
G6Pase 3
G6Pase-beta
Glucose 6 phosphatase, catalytic, 3
Glucose-6-phosphatase 3
Glucose-6-phosphatase beta
SCN4
Ubiquitous glucose-6-phosphatase catalytic subunit-related protein
UGRP
more
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Fig1: Sample:
A549(Human) Cell Lysate at 30 ug
HepG2(Human) Cell Lysate at 30 ug
Primary: Anti- G6PC3at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 39 kD
Observed band size: 39 kD
Fig2: Sample:
Muscle (Mouse) Lysate at 40 ug
Primary: Anti-G6PC3 at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 39 kD
Observed band size: 39 kD
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