| Type: |
Conjugated Primary Antibody |
| Conjugated with: |
594, ALEXA FLUOR® |
| Host organism: |
Rabbit (Oryctolagus cuniculus) |
| Target Protein/Peptide: |
HEXA/Beta hexosaminidase subunit alpha |
| Specificity: |
This antibody reacts specifically with HEXA/Beta hexosaminidase subunit alpha |
| Modification: |
No modification has been applied to this antibody |
| Modification site: |
None |
| Clonality: |
Polyclonal Antibody |
| Clone: |
Polyclonal Antibodies |
| Concentration: |
1ug per 1ul |
| Antigen Source: |
KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha |
| Applications: |
IF(IHC-P) |
| Applications with corresponding dilutions: |
IF(IHC-P)(1:50-200) |
| Cross reactive species: |
Mouse (Mus musculus), Rat (Rattus norvegicus), Human (Homo sapiens) |
| Cross Reactive Species details: |
However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species, No significant cross reactivity has been observed for this antibody for the tested species |
| Background information: |
A mutation in the &, HEXA, TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides, The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS, along with the cofactor CM2 activator protein, also designated beta-Hexosaminidase A, also known as GM2-gangliosidosis type I, catalyzes the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines in the brain and other tissues, chain, is a trimer composed of one &, one â, subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), &circ, &circ, «, «, -A chain and one â, -B chain and is found in the lysosomes of cells, Hexosaminidase A (HEXA), aring, aring |
| Purification method: |
Purified by Protein A |
| Storage: |
50% glycerol and 0, Store at 4°, 09% sodium azide, C for 12 months, Water buffered solution containing 100ug/ml BSA |
| Excitation emission: |
590nm/617nm |
| Also known as: |
HEXA/Beta hexosaminidase subunit alpha Antibody |
| Other name: |
Anti-HEXA/Beta hexosaminidase subunit alpha |
| Advisory: |
For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube, Prior to use briefly centrifuge the vial to gather all the solution on the bottom, specificity and sensitivity, thus reducing its reactivity, Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody |
| Properties: |
For facs or microscopy Alexa 1 conjugate |
| Conjugation: |
Alexa Fluor |
| Gene target: |
HEXA/Beta hexosaminidase subunit alpha |
| Short name: |
Fluor 594, Anti-HEXA/Beta hexosaminidase subunit alpha |
| Label: |
ALEXA |
| Alternative name: |
ALEXA Fluor 594, antibody to-HEXA/b hexosaminidase functionnal sequence a |
| Identity: |
4878 |
| Gene: |
HEXA |
More about : HEXA |
| Long gene name: |
hexosaminidase subunit alpha |
| Synonyms gene name: |
hexosaminidase A (alpha polypeptide) |
| Synonyms name: |
Tay Sachs disease GM2 gangliosidosis beta-hexosaminidase subunit alpha |
| Locus: |
15q23 |
| Discovery year: |
2001-06-22 |
| GenBank acession: |
M13520 |
| Entrez gene record: |
3073 |
| Pubmed identfication: |
2952641 3013851 |
| RefSeq identity: |
NM_000520 |
| Havana BLAST/BLAT: |
OTTHUMG00000133445 |
| Locus Specific Databases: |
Hexosaminidase A, Tay-Sachs Disease ALSOD, the Amyotrophic Lateral Sclerosis Online Genetic Database |