null

Anti-OTC Antibody (CAB9834)

SKU:
CAB9834
Product Type:
Antibody
Applications:
WB
IHC
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Metabolism
  • Metabolism Antibodies 3 Anti-OTC Antibody CAB9834
  • Metabolism Antibodies 3 Anti-OTC Antibody CAB9834
  • Metabolism Antibodies 3 Anti-OTC Antibody CAB9834
  • Metabolism Antibodies 3 Anti-OTC Antibody CAB9834
€139 - €419
Frequently bought together:

Description

system_update_altDatasheetsystem_update_altMSDS
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
Anti-OTC Polyclonal Antibody (CAB9834)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.
Anti-OTC Polyclonal Antibody (CAB9834)Immunohistochemistry of paraffin-embedded human stomach using OTC antibody (CAB9834) at dilution of 1:100 (40x lens).
Anti-OTC Polyclonal Antibody (CAB9834)Immunohistochemistry of paraffin-embedded human prostate using OTC antibody (CAB9834) at dilution of 1:100 (40x lens).
Anti-OTC Polyclonal Antibody (CAB9834)Immunohistochemistry of paraffin-embedded human liver damage using OTC antibody (CAB9834) at dilution of 1:100 (40x lens).

Related Products