Metabolism Antibodies 1
Anti-FA2H Antibody (CAB13874)
- SKU:
- CAB13874
- Product Type:
- Antibody
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
Antibody Name: | Anti-FA2H Antibody |
Antibody SKU: | CAB13874 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB |
Reactivity: | Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 95-170 of human FA2H (NP_077282.3). |
Application: | WB |
Recommended Dilution: | WB 1:500 - 1:2000 |
Reactivity: | Mouse, Rat |
Positive Samples: | Mouse brain, Mouse stomach, Mouse kidney, Rat brain |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 95-170 of human FA2H (NP_077282.3). |
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: | NEPV ALEE TQKT DPAM EPRF KVVD WDKD LVDW RKPL LWQV GHLG EKYD EWVH QPVT RPIR LFHS DLIE GLSK TVWY |
Gene ID: | 79152 |
Uniprot: | Q7L5A8 |
Cellular Location: | Endoplasmic reticulum membrane, Microsome membrane, Multi-pass membrane protein |
Calculated MW: | 18kDa/42kDa |
Observed MW: | 40kDa |
Synonyms: | FA2H, FAAH, FAH1, FAXDC1, SCS7, SPG35 |
Background: | This gene encodes a protein that catalyzes the synthesis of 2-hydroxysphingolipids, a subset of sphingolipids that contain 2-hydroxy fatty acids. Sphingolipids play roles in many cellular processes and their structural diversity arises from modification of the hydrophobic ceramide moiety, such as by 2-hydroxylation of the N-acyl chain, and the existence of many different head groups. Mutations in this gene have been associated with leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia. |
UniProt Protein Function: | FA2H: Required for alpha-hydroxylation of free fatty acids and the formation of alpha-hydroxylated sphingolipids. Defects in FA2H are a cause of spastic paraplegia autosomal recessive type 35 (SPG35). Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Belongs to the sterol desaturase family. SCS7 subfamily. |
UniProt Protein Details: | Protein type:Membrane protein, multi-pass; EC 1.-.-.-; Membrane protein, integral; Oxidoreductase Chromosomal Location of Human Ortholog: 16q23 Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane Biological Process: fatty acid metabolic process; sphingolipid biosynthetic process Disease: Spastic Paraplegia 35, Autosomal Recessive |
NCBI Summary: | This gene encodes a protein that catalyzes the synthesis of 2-hydroxysphingolipids, a subset of sphingolipids that contain 2-hydroxy fatty acids. Sphingolipids play roles in many cellular processes and their structural diversity arises from modification of the hydrophobic ceramide moiety, such as by 2-hydroxylation of the N-acyl chain, and the existence of many different head groups. Mutations in this gene have been associated with leukodystrophy dysmyelinating with spastic paraparesis with or without dystonia.[provided by RefSeq, Mar 2010] |
UniProt Code: | Q7L5A8 |
NCBI GenInfo Identifier: | 74749893 |
NCBI Gene ID: | 79152 |
NCBI Accession: | Q7L5A8.1 |
UniProt Secondary Accession: | Q7L5A8,O75213, Q96DK1, Q9H1A5, B7Z8T6, |
UniProt Related Accession: | Q7L5A8 |
Molecular Weight: | 18,324 Da |
NCBI Full Name: | Fatty acid 2-hydroxylase |
NCBI Synonym Full Names: | fatty acid 2-hydroxylase |
NCBI Official Symbol: | FA2H |
NCBI Official Synonym Symbols: | FAAH; FAH1; SCS7; SPG35; FAXDC1 |
NCBI Protein Information: | fatty acid 2-hydroxylase |
UniProt Protein Name: | Fatty acid 2-hydroxylase |
UniProt Synonym Protein Names: | Fatty acid alpha-hydroxylase |
Protein Family: | Fatty acid 2-hydroxylase |
UniProt Gene Name: | FA2H |
UniProt Entry Name: | FA2H_HUMAN |
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