PGM1 Recombinant Rabbit Monoclonal Antibody [JE65-06]
Catalog# HA721110
PGM1 Recombinant Rabbit Monoclonal Antibody [JE65-06]
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WB
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IF-Cell
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FC
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Human
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Mouse
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Rat
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Monkey
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unconjugated
概述
产品名称
PGM1 Recombinant Rabbit Monoclonal Antibody [JE65-06]
抗体类型
Recombinant Rabbit monoclonal Antibody
免疫原
Synthetic peptide within human PGM1 aa 513-562/562.
种属反应性
Human, Mouse, Rat, Monkey
验证应用
WB, IF-Cell, FC
分子量
Predicted band size: 61 kDa
阳性对照
A431 cell lysate, HeLa cell lysate, HepG2 cell lysate, NIH/3T3 cell lysate, COS-1 cell lysate, Mouse spleen tissue lysate, 293T cell lysate, Hela cell lysate, RAW264.7 cell lysate, PC-12 cell lysate, Rat spleen tissue lysate, A431, PC-12.
偶联
unconjugated
克隆号
JE65-06
RRID
产品特性
形态
Liquid
浓度
存放说明
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). It is recommended to aliquot into single-use upon delivery. Store at -20℃ long term.
存储缓冲液
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
亚型
IgG
纯化方式
Protein A affinity purified.
应用稀释度
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WB
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1:1,000-1:5,000
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IF-Cell
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1:100
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FC
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1:1,000
靶点
功能
Phosphoglucomutase-1 is an enzyme that in humans is encoded by the PGM1 gene. The protein encoded by this gene is an isozyme of phosphoglucomutase (PGM) and belongs to the phosphohexose mutase family. There are several PGM isozymes, which are encoded by different genes and catalyze the transfer of phosphate between the 1 and 6 positions of glucose. In most cell types, this PGM isozyme is predominant, representing about 90% of total PGM activity. In red blood cells, PGM2 is a major isozyme. This gene is highly polymorphic. Mutations in this gene cause CDG syndrome type 1t (CDG1T, formerly known as glycogen storage disease type XIV). Alternatively spliced transcript variants encoding different isoforms have been identified in this gene. Phosphoglucomutase 1 (PGM1) deficiency is an inherited metabolic disorder in humans (CDG syndrome type 1t, CDG1T). Affected patients show multiple disease phenotypes, including dilated cardiomyopathy, exercise intolerance, and hepatopathy, reflecting the central role of the enzyme in glucose metabolism.
背景文献
1. Altassan R. et. al. International consensus guidelines for phosphoglucomutase 1 deficiency (PGM1-CDG): Diagnosis, follow-up, and management. J Inherit Metab Dis. 2021 Jan
2. Radenkovic S. et. al. The Metabolic Map into the Pathomechanism and Treatment of PGM1-CDG. Am J Hum Genet. 2019 May
亚细胞定位
Cytoplasm.
别名
CDG1T antibody
Glucose phosphomutase 1 antibody
GSD14 antibody
OTTHUMP00000010519 antibody
OTTHUMP00000010520 antibody
PGM 1 antibody
PGM1 antibody
PGM1_HUMAN antibody
Phosphoglucomutase 1 antibody
Phosphoglucomutase-1 antibody
展开图片
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Western blot analysis of PGM1 on different lysates with Rabbit anti-PGM1 antibody (HA721110) at 1/5,000 dilution.
Lane 1: A431 cell lysate (20 µg/Lane)
Lane 2: HeLa cell lysate (20 µg/Lane)
Lane 3: HepG2 cell lysate (20 µg/Lane)
Lane 4: NIH/3T3 cell lysate (20 µg/Lane)
Lane 5: COS-1 cell lysate (20 µg/Lane)
Lane 6: Mouse spleen tissue lysate (30 µg/Lane)
Predicted band size: 61 kDa
Observed band size: 61 kDa
Exposure time: 6 seconds; ECL: K1801;
4-20% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (HA721110) at 1/5,000 dilution was used in primary antibody dilution (K1803) at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature. -
☑ Knockdown (KD)
Western blot analysis of PGM1 on different lysates with Rabbit anti-PGM1 antibody (HA721110) at 1/1,000 dilution.
Lane 1: HAP1-parental cell lysate
Lane 2: HAP1-PGM1 KD cell lysate
Lysates/proteins at 10 µg/Lane.
Predicted band size: 61 kDa
Observed band size: 61 kDa
Exposure time: 20 seconds; ECL: K1802;
4-20% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (HA721110) at 1/1,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature. -
Western blot analysis of PGM1 on different lysates with Rabbit anti-PGM1 antibody (HA721110) at 1/1,000 dilution.
Lane 1: 293T cell lysate, 10 µg/Lane
Lane 2: A431 cell lysate, 10 µg/Lane
Lane 3: Hela cell lysate, 10 µg/Lane
Lane 4: HepG2 cell lysate, 10 µg/Lane
Lane 5: NIH/3T3 cell lysate, 10 µg/Lane
Lane 6: RAW264.7 cell lysate, 10 µg/Lane
Lane 7: PC-12 cell lysate, 10 µg/Lane
Lane 8: Rat spleen tissue lysate, 20 µg/Lane
Predicted band size: 61 kDa
Observed band size: 61 kDa
Exposure time: 2 minutes;
10% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (HA721110) at 1/1,000 dilution was used in 5% NFDM/TBST at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature. -
Immunocytochemistry analysis of A431 cells labeling PGM1 with Rabbit anti-PGM1 antibody (HA721110) at 1/100 dilution.
Cells were fixed in 4% paraformaldehyde for 15 minutes at room temperature, permeabilized with 0.1% Triton X-100 in PBS for 15 minutes at room temperature, then blocked with 1% BSA in 10% negative goat serum for 1 hour at room temperature. Cells were then incubated with Rabbit anti-PGM1 antibody (HA721110) at 1/100 dilution in 1% BSA in PBST overnight at 4 ℃. Goat Anti-Rabbit IgG H&L (iFluor™ 488, HA1121) was used as the secondary antibody at 1/1,000 dilution. PBS instead of the primary antibody was used as the secondary antibody only control. Nuclear DNA was labelled in blue with DAPI.
Beta tubulin (HA601187, red) was stained at 1/100 dilution overnight at +4℃. Goat Anti-Mouse IgG H&L (iFluor™ 594, HA1126) was used as the secondary antibody at 1/1,000 dilution. -
Immunocytochemistry analysis of PC-12 cells labeling PGM1 with Rabbit anti-PGM1 antibody (HA721110) at 1/100 dilution.
Cells were fixed in 4% paraformaldehyde for 15 minutes at room temperature, permeabilized with 0.1% Triton X-100 in PBS for 15 minutes at room temperature, then blocked with 1% BSA in 10% negative goat serum for 1 hour at room temperature. Cells were then incubated with Rabbit anti-PGM1 antibody (HA721110) at 1/100 dilution in 1% BSA in PBST overnight at 4 ℃. Goat Anti-Rabbit IgG H&L (iFluor™ 488, HA1121) was used as the secondary antibody at 1/1,000 dilution. PBS instead of the primary antibody was used as the secondary antibody only control. Nuclear DNA was labelled in blue with DAPI.
Beta tubulin (HA601187, red) was stained at 1/100 dilution overnight at +4℃. Goat Anti-Mouse IgG H&L (iFluor™ 594, HA1126) was used as the secondary antibody at 1/1,000 dilution. -
Flow cytometric analysis of A431 cells labeling PGM1.
Cells were fixed and permeabilized. Then stained with the primary antibody (HA721110, 1/1,000) (red) compared with Rabbit IgG Isotype Control (green). After incubation of the primary antibody at +4℃ for an hour, the cells were stained with a iFluor™ 488 conjugate-Goat anti-Rabbit IgG Secondary antibody (HA1121) at 1/1,000 dilution for 30 minutes at +4℃. Unlabelled sample was used as a control (cells without incubation with primary antibody; black). -
Flow cytometric analysis of PC-12 cells labeling PGM1.
Cells were fixed and permeabilized. Then stained with the primary antibody (HA721110, 1/1,000) (red) compared with Rabbit IgG Isotype Control (green). After incubation of the primary antibody at +4℃ for an hour, the cells were stained with a iFluor™ 488 conjugate-Goat anti-Rabbit IgG Secondary antibody (HA1121) at 1/1,000 dilution for 30 minutes at +4℃. Unlabelled sample was used as a control (cells without incubation with primary antibody; black).
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