Item no. |
STJ116741-50 |
Manufacturer |
St John's Laboratory
|
Amount |
50ul |
Category |
|
Type |
Antibody Polyclonal |
Applications |
WB |
Specific against |
Human, Mouse, Rat |
Host |
Rabbit |
Isotype |
IgG |
ECLASS 10.1 |
32160702 |
ECLASS 11.0 |
32160702 |
UNSPSC |
12352203 |
Alias |
Anti-Dihydrolipoyllysine-Residue Acetyltransferase Component Of Pyruvate Dehydrogenase Complex-Mitochondrial antibody</br>Anti-70 Kda Mitochondrial Autoantigen Of Primary Biliary Cirrhosis antibody</br>Anti-Pbc antibody</br>Anti-Dihydrolipoamide Acetyltra |
Similar products |
Anti-Dihydrolipoyllysine-Residue Acetyltransferase Component Of Pyruvate Dehydrogenase Complex-Mitochondrial antibodyAnti-70 Kda Mitochondrial Autoantigen Of Primary Biliary Cirrhosis antibodyAnti-Pbc antibodyAnti-Dihydrolipoamide Acetyltra |
Available |
|
Clonality |
Polyclonal |
Conjugation |
Unconjugated |
Formulation |
PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification |
Affinity purification |
Uniprot ID |
P10515 |
Storage |
Store at-20° C and avoid freeze-thaw cycles. |
Description |
This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood. |
Gene ID |
|
Gene symbol |
|
Immunogen |
Recombinant fusion protein containing a sequence corresponding to amino acids 87-270 of human DLAT (NP_001922. 2). |
Short Description |
Rabbit polyclonal against DLAT |
Cellular localization |
Mitochondrion Matrix |
Molecular weight |
Calculated: 68kDa. Observed: 69kDa |
Dilution range |
WB 1:500-1:2000 |
Alternative names |
Anti-Dihydrolipoyllysine-Residue Acetyltransferase Component Of Pyruvate Dehydrogenase Complex-Mitochondrial antibodyAnti-70 Kda Mitochondrial Autoantigen Of Primary Biliary Cirrhosis antibodyAnti-Pbc antibodyAnti-Dihydrolipoamide Acetyltransferase Component Of Pyruvate Dehydrogenase Complex antibodyAnti-M2 Antigen Complex 70 Kda Subunit antibodyAnti-Pyruvate Dehydrogenase Complex Component E2 antibodyAnti-Pdc-E2 antibodyAnti-Pdce2 antibodyAnti-DLAT DLTA antibody |
Protein name |
Dihydrolipoyllysine-Residue Acetyltransferase Component Of Pyruvate Dehydrogenase Complex-Mitochondrial70 Kda Mitochondrial Autoantigen Of Primary Biliary CirrhosisPbcDihydrolipoamide Acetyltransferase Component Of Pyruvate Dehydrogenase ComplexM2 Antigen Complex 70 Kda SubunitPyruvate Dehydrogenase Complex Component E2Pdc-E2Pdce2 |
Note |
FOR RESEARCH USE ONLY (RUO). |
Swiss port key |
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Note: The presented information and documents (Manual, Product Datasheet, Safety Datasheet and Certificate of Analysis) correspond to our latest update and should serve for orientational purpose only. We do not guarantee the topicality. We would kindly ask you to make a request for specific requirements, if necessary.
All products are intended for research use only (RUO). Not for human, veterinary or therapeutic use.