Metabolism Antibodies 1
Anti-FAH Antibody (CAB13492)
- SKU:
- CAB13492
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-FAH Antibody |
| Antibody SKU: | CAB13492 |
| 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 1-419 of human FAH (NP_000128.1). |
| Application: | WB IHC |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | Mouse liver, Mouse kidney, Rat liver, Rat kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-419 of human FAH (NP_000128.1). |
| 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: | MSFI PVAE DSDF PIHN LPYG VFST RGDP RPRI GVAI GDQI LDLS IIKH LFTG PVLS KHQD VFNQ PTLN SFMG LGQA AWKE ARVF LQNL LSVS QARL RDDT ELRK CAFI SQAS ATMH LPAT IGDY TDFY SSRQ HATN VGIM FRDK ENAL MPNW LHLP VGYH GRAS SVVV SGTP IRRP MGQM KPDD SKPP VYGA CKLL DMEL EMAF FVGP GNRL GEPI PISK AHEH IFGM VLMN DWSA RDIQ KWEY VPLG PFLG KSFG TTVS PWVV PMDA LMPF AVPN PKQD PRPL PYLC HDEP YTFD INLS VNLK GEGM SQAA TICK SNFK YMYW TMLQ QLTH HSVN GCNL RPGD LLAS GTIS GPEP ENFG SMLE LSWK GTKP IDLG NGQT RKFL LDGD EVII TGYC QGDG YRIG FGQC AGKV LPAL LPS |
| Gene ID: | 2184 |
| Uniprot: | P16930 |
| Cellular Location: | |
| Calculated MW: | 38kDa/46kDa |
| Observed MW: | 46kDa |
| Synonyms: | FAH |
| Background: | This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). |
| UniProt Protein Function: | FAH: Defects in FAH are the cause of tyrosinemia type 1 (TYRO1). An inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include hepatic necrosis, renal tubular injury, episodic weakness, self-mutilation, and seizures. Renal tubular dysfunction is associated with phosphate loss and hypophosphataemic rickets. Progressive liver disease can lead to the development of hepatocellular carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment. Belongs to the FAH family. |
| UniProt Protein Details: | Protein type:Amino Acid Metabolism - tyrosine; EC 3.7.1.2; Hydrolase Chromosomal Location of Human Ortholog: 15q25.1 Cellular Component: cytosol Molecular Function:fumarylacetoacetase activity; metal ion binding Biological Process: L-phenylalanine catabolic process; tyrosine catabolic process; arginine catabolic process Disease: Tyrosinemia, Type I |
| NCBI Summary: | This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). [provided by RefSeq, Jul 2008] |
| UniProt Code: | P16930 |
| NCBI GenInfo Identifier: | 119778 |
| NCBI Gene ID: | 2184 |
| NCBI Accession: | P16930.2 |
| UniProt Related Accession: | P16930 |
| Molecular Weight: | ~ 46kDa |
| NCBI Full Name: | Fumarylacetoacetase |
| NCBI Synonym Full Names: | fumarylacetoacetate hydrolase |
| NCBI Official Symbol: | FAH |
| NCBI Protein Information: | fumarylacetoacetase |
| UniProt Protein Name: | Fumarylacetoacetase |
| UniProt Synonym Protein Names: | Beta-diketonase; Fumarylacetoacetate hydrolase |
| Protein Family: | Fumarylacetoacetase |
| UniProt Gene Name: | FAH |
| UniProt Entry Name: | FAAA_HUMAN |
 | Western blot analysis of extracts of various cell lines, using FAH antibody (CAB13492) 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: 5s. |
 | Immunohistochemistry of paraffin-embedded rat liver using FAH antibody (CAB13492) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded rat kidney using FAH antibody (CAB13492) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human liver damage using FAH antibody (CAB13492) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse liver using FAH antibody (CAB13492) at dilution of 1:100 (40x lens). |
View AllClose
Related Products
Fumarylacetoacetase/FAH Recombinant Protein (RPES4423)
Metabolism Recombinant Proteins
system_update_altDatasheetFumarylacetoacetase/FAH Recombinant Protein Fumarylacetoacetase belongs to the FAH family. Fumarylacetoacetase is primary expressed in liver and kidney. It exists as a h
Human FAH ELISA Kit (HUFI07933)
system_update_altDatasheet - तकनीकी मैनुअलsystem_update_altMSDS - तकनीकी
