2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
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产品名称: 2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
产品型号: abx025837
产品展商: 其他品牌
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简单介绍
2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
的详细介绍
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems.
| Target |
BCKDHB |
| Reactivity |
Human |
| Host |
Rabbit |
| Clonality |
Polyclonal |
| Tested Applications |
WB, IHC, IF/ICC, FCM |
| Recommended dilutions |
Optimal dilutions/concentrations should be determined by the end user. |
| Immunogen |
Human BCKDHB. |
| Purification |
Peptide Affinity Purified Rabbit Polyclonal Antibody. |
| Isotype |
IgG |
| Conjugation |
Unconjugated |
| Specificity |
This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB. |
| Storage |
Aliquot and store at -20 °C. Avoid repeated freeze/thaw cycles. |
| Swiss Prot |
P21953
|
| Gene Symbol |
BCKDHB |
| Buffer |
PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
| UNSPSC Code |
12352203 |
| Availability |
Shipped within 5-10 working days. |
| Note |
This product is for research use only. |
