Cell Biology Antibodies 4
Anti-BTD Antibody (CAB13955)
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- SKU:
- CAB13955
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
| Antibody Name: | Anti-BTD Antibody |
| Antibody SKU: | CAB13955 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 284-543 of human BTD (NP_000051.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | Jurkat, Mouse liver, Rat kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 284-543 of human BTD (NP_000051.1). |
| 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: | IQKA FAVA FGIN VLAA NVHH PVLG MTGS GIHT PLES FWYH DMEN PKSH LIIA QVAK NPVG LIGA ENAT GETD PSHS KFLK ILSG DPYC EKDA QEVH CDEA TKWN VNAP PTFH SEMM YDNF TLVP VWGK EGYL HVCS NGLC CYLL YERP TLSK ELYA LGVF DGLH TVHG TYYI QVCA LVRC GGLG FDTC GQEI TEAT GIFE FHLW GNFS TSYI FPLF LTSG MTLE VPDQ LGWE NDHY FLRK SRLS SGLV TAAL YGRL YERD |
| Gene ID: | 686 |
| Uniprot: | P43251 |
| Cellular Location: | Secreted, extracellular space |
| Calculated MW: | 58kDa/61kDa |
| Observed MW: | 65-70kDa |
| Synonyms: | BTD |
| Background: | The protein encoded by this gene functions to recycle protein-bound biotin by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. The encoded protein has also been shown to have biotinyl transferase activity. Mutations in this gene are associated with biotinidase deficiency. Multiple transcript variants encoding different isoforms have been described. |
| UniProt Protein Function: | BTD: Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. Defects in BTD are the cause of biotinidase deficiency (BTD deficiency); also called late-onset multiple carboxylase deficiency. BTD deficiency is a juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis. BTD deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur. Belongs to the CN hydrolase family. BTD/VNN subfamily. |
| UniProt Protein Details: | Protein type:EC 3.5.1.12; Ligase; Secreted, signal peptide; Cofactor and Vitamin Metabolism - biotin; Hydrolase; Secreted Chromosomal Location of Human Ortholog: 3p25 Cellular Component: extracellular space; mitochondrial matrix; apical part of cell; extracellular region; nucleolus; perikaryon Molecular Function:biotinidase activity; biotin carboxylase activity Biological Process: epidermis development; central nervous system development; vitamin metabolic process; biotin metabolic process; water-soluble vitamin metabolic process Disease: Biotinidase Deficiency |
| NCBI Summary: | The protein encoded by this gene functions to recycle protein-bound biotin by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. The encoded protein has also been shown to have biotinyl transferase activity. Mutations in this gene are associated with biotinidase deficiency. Multiple transcript variants encoding different isoforms have been described. [provided by RefSeq, Aug 2013] |
| UniProt Code: | P43251 |
| NCBI GenInfo Identifier: | 226693503 |
| NCBI Gene ID: | 686 |
| NCBI Accession: | P43251.2 |
| UniProt Secondary Accession: | P43251,Q96EM9, A6NHF2, B2R865, B4DFX1, B4DLJ9, B7Z7C9 F8W1Q3, |
| UniProt Related Accession: | P43251 |
| Molecular Weight: | 543 |
| NCBI Full Name: | Biotinidase |
| NCBI Synonym Full Names: | biotinidase |
| NCBI Official Symbol: | BTD |
| NCBI Protein Information: | biotinidase; biotinase |
| UniProt Protein Name: | Biotinidase |
| Protein Family: | Biotinidase |
| UniProt Gene Name: | BTD |
| UniProt Entry Name: | BTD_HUMAN |
 | Western blot analysis of extracts of various cell lines, using BTD antibody (CAB13955) 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: 90s. |
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