In D glucose, carbons 4, 5, and 6 are derived from
D-glyceraldehyde
At equilibrium, is most glucose in the cyclic form of the open chain form?
cyclic
In beta d glucopyranose, the 1 hydroxyl is in the _____ position while in in alpha glucopyranose, the 1 hydroxyl is in the ____ position
up, down
The two steps of protein glycosylation are first the formation of a _____ base and then a _____
Schiff (imine), Amadori rearrangement to give you a amide
HbA1C is formed by glycation of an N terminal ____ in Hb and then it undergoes the usual rearrangement
valine on the Beta chain(HbA)
For alpha configurations, the C5 hydrogen and C1 hydroxyl should be pointing in the _____ direction
same
For beta sugar configurations, the C1 hydroxyl group and C5 hydrogen will be pointing in _____ direction
opposite
The C2 epimer of glucose is called
mannose
The C4 epimer of glucose is called
galactose
The C4 epimer of galactose is called
N-acetylgalactosamine
Glucouronic acid has a ____ group on carbon ____ while L Iduronic acid has the same thing pointed down
COOH, 6
Which enzyme cleaves neuraminic acids/sialic from its protein?
neuraminidase
Lactose is a combination of ____ linked ____ and ____
beta 1,4 glucose, galactose
The formation of the dolichol phosphate oligonucleotide precursor occurs in the ____, and modifications occur in the_____
ER, Golgi
In the presence of alpha lactalbumin, the enzyme ______ turn UDP galactose into _____
beta galactotransferase, lactose (acceptor is glucose)
In the absence of alpha lactaalbumin, the enzyme _____ turns UDP galactose into _____
beta galactotransferase, N-Acetyllactosamine (acceptor is n acetylglucosamine)
The most common O glycosidic linkage is between ______ and _____(sugar)
Ser/Thre, N-acetylgalactosamine
The most common N glycosidic linkage is between ____ and ____(sugar)
Asparagine, N-acetylglucosamine
The common protein backbone in N linked glycosylated backbones is _____
Asn,X, Ser/Thre
A complex(acidic type) N linked glycosylated structure contains which extra sugars in addition to the core
sialic acid, Nacetyl glucosamine, and galactose
The core sugar structure of any N linked structure has
2 GlucNac then 1 beta linked mannose, then 2 alpha linked mannoses
The first step of glycan biosynthesis involves the formation of a high mannose sugar and its attachment to ______. in the ER. How do you know its complete?
Dolichol phosphate, addition of 3 glucoses
The processing of high mannose to complex type occurs in the _____
ER and Golgi and involves glycosidases
The Amadorri reaction is (fast/slow)
slow
The flu virus has 2 viral coat protein each with 16 and 9 subtypes.
Hemagglutinin, Neuramidase
Hemagglutinin (virus) binds to _____ on respiratory epithelial, which Neuramidase
sialic, cleaves sialic acid so that virus can escape
Which 2 drugs are neuramidase inhibitors used to treat Influenza A
Oseltamivir (Tamiflu) Zanamvir (Relenza)
Condroitin sulfate proteoglycans are called_____ and it involves condroitin sulfate and keratin sulfate GAGS linked to a core protein via ______
Serine Xylose-Galactose-Glucose
Which is the only GAG not linked to a core protein?
hyaluronic acid
Hyaluronic acid is comprised of ____ and _____ joined by a Beta __,___ linkage
Glucouronic acid, N-Acetylglucosamine, beta 1,3
Hyaluronic acid is important in the
eye, synovial fluid of joints
Chondroitin-4-sulfate is comprised of ____ and ____ and its important in _______
Glucouronic acid, N-Acetylgalactosamine, cartilidge, tendons, ligaments
Dermatan sulfate is comprised of ___ and _____ through a ____ linkage and its found in _____
L-iduronic acid, N-acetylgalactosamine, alpha 1,3, skin, blood vessels
Keratin Sulfate composed of ____ and ____ and is found in _____
galactose, N-acetylglucosamine (beta 1,4 then beta 1,3 to form N-acetyllactossamine), cartilage and cornea
Heparin is an ____ component of mast and liver cells while Heparin sulfate is ____
intracellular, extracellular
Heparin and heparin sulfate are composed of ____
L-idouronic acid or d glucouronic acid and d-glucosamine
The only alpha 1,4 linkage in any GAG is in _____
L-idouronic acid to d glucosamine, heparin
Heparin binds to ______ which leads to thrombin inactivation
antithrombin III
Gram positive bacteria have a ______ while gram negative bacteria have a ____
thick peptidoglycan layer external to the pm, thin peptidoglycan layer between the pm and outer membrane
The sugars in a peptidoglycan are ___ and ____
Beta 1,4 N acetyl glucosamine, N acetyl muramic acid
The tetrapeptide side chain in a peptidoglycan is_____and the 5 glycines are attached to the
L alanine, D glutamate, L lysine, D alanine, L lysine
Cross linking of strands is done from the glycines to the ______ by _____
D alanine, transpeptidase
What inhibits transpeptidases?
Beta lactams, penicillin
Hunter syndrome is caused by a deficiency in _____ leading to a build up of ___ and ____
Iduronate sulfatase, dermatan sulfate, heparin sulfate
After the sulfate is cleaved off the terminal ends of DS and HS, the enzyme ____ removes Iduronic acid and its deficiency is ______
alpha L Iduronidase, Hurler syndrome, DS and HS buildup
Sanfilippos type A is caused by a deficiency in ____ leading to a buidup of
Heparan N sulfatase heparin sulfate with modifications,
Sanfilippos type C is caused by a deficiency in ______ and the buildup product is ____
Acetyl-CoA acetyl transferase, heparin sulfate with modifications
Sanfilippos type B is caused by a deficiency
N acetyl glucosaminidase, heparin sulfate with modifications
Morquio syndrome Type A is characterized by buildup in ______ because of loss of________
Keratin sulfate, Galactose 6 sulfatase
Morquio syndrome Type B is characterized by buildup in ________ because loss of ______
modified KS, beta galactosidase
Each glycosphingolipid contains a ____ and a ____
ceramide backbone, hydrophilic sugar chain
Glycosphingolipids can be used to regulate differentiation and _______, and ceramides are very important on the skin where they act as a ______
apoptosis, water permeability barrier
The major acidic glycosphingolipids are
gangliosides (contain sialic acid)
Galactosylceramide is found mainly in the _____ and a defect in its degradation causes _______
CNS Krabbe disease
Glucosylceramide is found mainly in ____ and a defect in its degradation causes _____
visceral organs, Gaucher's disease
Lactosylceramide is the addition of what 2 sugars
galactose and glucose via a beta 1,4 linkage
Globotriaosylceramide is found in ____ and the defect in its degradation leads to ____, and what is the additional sugar added
visceral organs, Fabry disease, galactose with an alpha 1,4 linkage
Globotetraosylceramide is found in _____ and the additional sugar added is _____ with a ___ linkage
visceral organs, n acetylgalactosamine, beta 1,3
The ganglioside (GM3) is the major ganglioside in _______ and it is made up of ______
visceral organs, lactosylceramide with N-acetyl neruaminic acid attached via an alpha 2,3 linkage
The ganglioside GM2 is associated with _____ disease and it consists of an additional _____
Tach-Sachs, N acetyl galactosamine via a beta 1,4 linkage
GM1 is the major ganglioside of the ____ and a receptor for the _____ and consists of an additional ______
CNS, cholera toxin, Galactose beta 1,3 linkage
GM1 gangliosidosis is caused by the accumulation of ____ and a deficiency in ____
GM1, beta galactosidase
The accumulation of GM2 is caused by the lack of the enzyme _______ in the presence of cofactor ______ which leads to the disease _____
Beta-Hexosaminidase A, GM2-activator, Tay Sachs
GM3 is catabolized via
Sialiase(Neuramidase) then beta galactosidase
The H antigen defines blood group ____made up of a ______
O, fucose-galactose chain with alpha 1-2 linkage
If a galactose is attached to the H group, the antigen is
Beta determinant
If a GalNac is attached to the H group, the antigen is a
Alpha determinant
Gaucher, Krabbe, and Tay-Sachs are transmitted in a ____ manner, while Fabrey's is _____
autosomal recessive, X-linked
Fabry disease is a disorder of ___ catabolism and the deficient enzyme is _____
Gb3, alpha galactosidase A
Gaucher's disease is caused by a deficiency in _____, which allows glucoceramide to accumulate in ____
glucocerebrosidase, tissue macrophages
Gaucher's Type 1 is _____ while Type 2 and 3 are
adult non neuropathic and can be treated, non treatable for brain damage
Krabbe disease causes _____ and is caused by a deficiency in _____
CNS + peripheral nerve demyleination, galactocerebrosidase (lysosomal)
Does galactocerebroside accumulate in the brain with Krabbe disease? What does?
NO, Galactosylsphingosine (psychosine) as the enzyme galactocerebrosidase degrades both and psychosine is normally present at low levels builds up destroying oligodendrocytes
Classic Tay Sach's disease is caused by lack of ____
Beta-hexoaminidase A because this hydrolyzes the terminal GalNAc in GM2
A mutation in the alpha chain only affects the enzyme____ and thus they have the variant _____
Hex A, Variant B(classic Tay-Sachs) and can't digest GM2
A mutation in the beta chain has problems digesting ______ because they lack ______ and this is called ____
Gb4, GM2, Hex A and Hex B, Tay Sachs Varient O
Tay-Sachs variant AB is when they lack ______ and thus can digest _____ but not ______
GM2 activator, Gb4, GM2
Universal donors are ____ and universal recipients are ____
Type O, Type AB
The enzyme replacement for hunters disease is
Elaprase
Hurlers syndrome can be treated by
Aldurazyme
Which contains more ido-a and n and o sulfated glucosamine, heparin or heparin sulfate?
Heparin
Hyaluronic acid is ___ long while other gags have 20-60 repeats
50,000
Fabry disease causes accumulation of gb3 in
Kidney, heart , skin burning pain
The pain in fabrys will respond to
Anti convulsants
The major ganglioside in the cns is
Gm3
Sanfillipo syndrome is ______ only while morquio is ____ only
Neurological, somatic
Sanfillipo syndrome is ______ only while morquio is ____ only
Neurological, somatic