Anti-OTC Antibody (CAB9834)
- SKU:
- CAB9834
- Product type:
- Antibody
- Application:
- WB
- Application:
- IHC
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Metabolism
Frequently bought together:
Description
| Antibody Name: | Anti-OTC Antibody |
| Antibody SKU: | CAB9834 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 215-354 of human OTC (NP_000522.3). |
| Application: | WB IHC |
| Recommended Dilution: | WB 1:1000 - 1:4000 IHC 1:50 - 1:100 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | Mouse liver, Mouse small intestine, Rat liver, Rat small intestine |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 215-354 of human OTC (NP_000522.3). |
| 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: | LQAA TPKG YEPD ASVT KLAE QYAK ENGT KLLL TNDP LEAA HGGN VLIT DTWI SMGQ EEEK KKRL QAFQ GYQV TMKT AKVA ASDW TFLH CLPR KPEE VDDE VFYS PRSL VFPE AENR KWTI MAVM VSLL TDYS PQLQ KPKF |
| Gene ID: | 5009 |
| Uniprot: | P00480 |
| Cellular Location: | Mitochondrion matrix |
| Calculated MW: | 39kDa |
| Observed MW: | 38kDa |
| Synonyms: | OTC, OCTD |
| Background: | This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. |
| UniProt Protein Function: | OTC: Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD). OTCD is an X- linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms. Belongs to the ATCase/OTCase family. |
| UniProt Protein Details: | Protein type:EC 2.1.3.3; Transferase; Amino Acid Metabolism - arginine and proline; Mitochondrial Chromosomal Location of Human Ortholog: Xp21.1 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:ornithine carbamoyltransferase activity Biological Process: arginine biosynthetic process via ornithine; citrulline biosynthetic process; ornithine catabolic process; urea cycle Disease: Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To |
| NCBI Summary: | This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P00480 |
| NCBI GenInfo Identifier: | 84028235 |
| NCBI Gene ID: | 5009 |
| NCBI Accession: | P00480.3 |
| UniProt Secondary Accession: | P00480,Q3KNR1, Q6B0I1, Q9NYJ5, A8K9P2, D3DWB0, |
| UniProt Related Accession: | P00480 |
| Molecular Weight: | 39,935 Da |
| NCBI Full Name: | Ornithine carbamoyltransferase, mitochondrial |
| NCBI Synonym Full Names: | ornithine carbamoyltransferase |
| NCBI Official Symbol: | OTC |
| NCBI Official Synonym Symbols: | OCTD |
| NCBI Protein Information: | ornithine carbamoyltransferase, mitochondrial |
| UniProt Protein Name: | Ornithine carbamoyltransferase, mitochondrial |
| UniProt Synonym Protein Names: | Ornithine transcarbamylase; OTCase |
| UniProt Gene Name: | OTC |
| UniProt Entry Name: | OTC_HUMAN |
 | Western blot analysis of extracts of various cell lines, using OTC antibody (CAB9834) 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 Basic Kit (RM00020). Exposure time: 1s. |
 | Immunohistochemistry of paraffin-embedded human stomach using OTC antibody (CAB9834) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human prostate using OTC antibody (CAB9834) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human liver damage using OTC antibody (CAB9834) at dilution of 1:100 (40x lens). |
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