Cell Biology Antibodies 7
Anti-GLA Antibody (CAB1700)
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- SKU:
- CAB1700
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
| Antibody Name: | Anti-GLA Antibody |
| Antibody SKU: | CAB1700 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC IF |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 150-429 of human GLA (NP_000160.1). |
| Application: | WB IHC IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100 |
| Reactivity: | Human, Mouse |
| Positive Samples: | SW620, BT-474, HeLa |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 150-429 of human GLA (NP_000160.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | GYYD IDAQ TFAD WGVD LLKF DGCY CDSL ENLA DGYK HMSL ALNR TGRS IVYS CEWP LYMW PFQK PNYT EIRQ YCNH WRNF ADID DSWK SIKS ILDW TSFN QERI VDVA GPGG WNDP DMLV IGNF GLSW NQQV TQMA LWAI MAAP LFMS NDLR HISP QAKA LLQD KDVI AINQ DPLG KQGY QLRQ GDNF EVWE RPLS GLAW AVAM INRQ EIGG PRSY TIAV ASLG KGVA CNPA CFIT QLLP VKRK LGFY EWTS RLRS HINP TGTV LLQL ENTM QMSL KDLL |
| Gene ID: | 2717 |
| Uniprot: | P06280 |
| Cellular Location: | Lysosome |
| Calculated MW: | 48kDa |
| Observed MW: | 49kDa |
| Synonyms: | GLA, GALA |
| Background: | This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. |
| UniProt Protein Function: | GLA: Defects in GLA are the cause of Fabry disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Belongs to the glycosyl hydrolase 27 family. |
| UniProt Protein Details: | Protein type:Lipid Metabolism - sphingolipid; Lipid Metabolism - glycerolipid; EC 3.2.1.22; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; Carbohydrate Metabolism - galactose Chromosomal Location of Human Ortholog: Xq22 Cellular Component: cytoplasm; extracellular region; Golgi apparatus; lysosomal lumen; lysosome Molecular Function:alpha-galactosidase activity; catalytic activity; hydrolase activity; protein binding; protein homodimerization activity; receptor binding Biological Process: glycosphingolipid catabolic process; glycosphingolipid metabolic process; glycosylceramide catabolic process; negative regulation of nitric oxide biosynthetic process; negative regulation of nitric-oxide synthase activity; oligosaccharide metabolic process Disease: Fabry Disease |
| NCBI Summary: | This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P06280 |
| NCBI GenInfo Identifier: | 113499 |
| NCBI Gene ID: | 2717 |
| NCBI Accession: | P06280.1 |
| UniProt Secondary Accession: | P06280,Q6LER7, |
| UniProt Related Accession: | P06280 |
| Molecular Weight: | 48,767 Da |
| NCBI Full Name: | Alpha-galactosidase A |
| NCBI Synonym Full Names: | galactosidase alpha |
| NCBI Official Symbol: | GLA |
| NCBI Official Synonym Symbols: | GALA |
| NCBI Protein Information: | alpha-galactosidase A |
| UniProt Protein Name: | Alpha-galactosidase A |
| UniProt Synonym Protein Names: | Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; INN: Agalsidase |
| Protein Family: | Glacontryphan |
| UniProt Gene Name: | GLA |
| UniProt Entry Name: | AGAL_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GLA antibody (CAB1700) at 1:1000 dilution._Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution._Lysates/proteins: 25ug per lane._Blocking buffer: 3% nonfat dry milk in TBST._Detection: ECL Enhanced Kit (RM00021)._Exposure time: 30s. |
 | Immunohistochemistry of paraffin-embedded human liver using GLA antibody (CAB1700) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse kidney using GLA antibody (CAB1700) at dilution of 1:100 (40x lens). |
 | Immunofluorescence analysis of HeLa cells using GLA antibody (CAB1700). Blue: DAPI for nuclear staining. |
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