Anti-HMGCL antibody
| 英文名称 | HMGCL |
| 中文名称 | 三羟基三甲基辅酶A裂解酶抗体 |
| 别 名 | 3 hydroJNC 3 methylglutaryl CoA lyase; 3 hydroJNC 3 methylglutaryl Coenzyme A lyase; 3 hydroJNCmethyl 3 methylglutaryl Coenzyme A lyase (hydroJNCmethylglutaricaciduria); 3 hydroJNCmethyl 3 methylglutaryl Coenzyme A lyase; 3-hydroJNC-3-methylglutarate-CoA lyase; HL; HMG CoA lyase; HMG CoA Lyase Deficiency; HMG-CoA lyase; HMGCL; HMGCL_HUMAN; HydroJNCmethylglutaricaciduria; HydroJNCmethylglutaryl CoA lyase; HydroJNCmethylglutaryl CoA lyase mitochondrial; HydroJNCmethylglutaryl-CoA lyase; mitochondrial; MS725; OTTHUMP00000044830. |
DATASHEET
Host:Rabbit
Target Protein:HMGCL
IR:Immunogen Range:41-140/325
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:3155
Swiss Prot:P35914
Source:KLH conjugated synthetic peptide derived from human HMGCL:41-140/325
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Background:HydroJNCmethylglutaryl-CoA lyase (HMGCL) is found in fibroblasts, liver and lymphoblasts. It has a role in ketogenesis and leucine catabolism. Defects in HMGCL are the cause of 3-hydroJNC-3-methylglutaryl-CoA lyase deficiency (hydroJNCmethylglutaricaciduria), an autosomal recessive disease which can lead to hypoglycemia and coma.
Size:100ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
Mouse
Rat
Chicken
Dog
Horse
Rabbit
.
For research use only. Not intended for diagnostic or therapeutic use.
VALIDATION IMAGES
Sample:
Liver (Mouse) Lysate at 40 ug
Primary: Anti- HMGCL (bs-5067R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 32 kD
Observed band size: 30 kD
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